Metastatic neuroblastoma diagnosed on prenatal sonographic examination performed for decreased fetal movement.

We report a case of fetal neuroblastoma presenting with massive liver metastasis diagnosed during the biophysical profile sonographic examination performed for decreased fetal movement. The patient presented at 37 weeks' gestation with limited fetal movement over 24 hours. Biophysical profile showed marked polyhydramnios and an enlarged abdomen filled with a homogeneous mass lesion suspicious for liver metastasis. Primary urgent cesarean section was performed revealing a cachectic neonate with a rigid and grossly distended abdomen. Neonatal evaluation confirmed the etiology of the abdominal mass to be liver metastasis from neuroblastoma. The child died on the 46th day. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:502-506, 2017.

Neuroblastoma in monozygotic twins with distinct presentation pathology and outcome: is it familial or in utero metastasis.

To date ten sets of monozygotic twins with neuroblastoma have been reported in the literature. Twin-to-twin in utero metastasis have been proposed as the mechanism of tumor development in the second twin; based on similar pathology, presence of metastatic disease, absence of a primary tumor, and/or later presentation in the second twin. Hereditary neuroblastoma has not been described in this context. We propose that primary neuroblastoma can occur in monozygotic twins without twin-twin transmission; due to the different ages of presentation, histology, ploidy, and tumor behavior.

Adrenal tumours are more predominant in females regardless of their histological subtype: a review.

OBJECTIVES: Adrenal tumours are a heterogeneous group of rare tumours. The aim of this article was to critically review gender-specific differences in the incidence, prognosis and symptoms of the different subtypes of adrenal tumours. METHODS: Data acquisition regarding gender differences in adrenal tumours was performed using MEDLINE searches with combinations of the following keywords: adrenal tumours, gender, sex differences, adrenocortical carcinoma, pheochromocytoma, incidentaloma, risk factors and genetic aspects. RESULTS: Data are scarce in the literature concerning the effects of gender on adrenal lesions. Although the incidence of most types of tumours (other than breast cancer and other gender-related tumours) is higher in men than in women, evidence suggests that adrenal tumours (i.e. incidentalomas, adrenal carcinomas, oncocytomas and adrenal cysts) are more frequent in women than in men. In addition, female patients have significantly increased numbers of self-reported signs and symptoms of pheochromocytoma than male patients, irrespective of biochemical phenotype and tumour presentation. Relatively little research has been performed examining the reasons for these disparities. However, hormonal interactions involving complex adrenal, endocrine and neurocrine functions together with variations in hormonal receptor sensitivity have been hypothesised to be involved. CONCLUSION: Gender differences exist in the incidence and symptoms of several subtypes of adrenal tumours. The reasons for these disparities are not well established. In addition to epidemiological data, these results need to be further investigated to better understand the role of genetic and hormonal predispositions in the development, behaviour and aggressiveness of adrenal tumours.

Concordance for neuroblastoma in monozygotic twins: case report and review of the literature.

The patients were infant male twins born by cesarean delivery following a healthy pregnancy at 36 weeks' gestation to unrelated parents. At 4 months of age, twin 2 presented with hepatomegaly and a right suprarenal mass. Resection of an adrenal tumor and a liver tumor biopsy were performed. Twin 1 had no symptoms at 4 months of age. Screening by abdominal ultrasonography showed multiple masses in the liver but no adrenal mass. Metaiodobenzylguanidine scintigraphy showed positive findings in multiple liver masses. A laparoscopic biopsy for a liver tumor was performed. All primary tumor and liver tumor specimens from twin 2 and the liver tumor of twin 1 had the same histologic classification of neuroblastoma and nearly identical genetic aberrations, including a chromosome gain or loss using array-comparative genomic hybridization. From these clinical and pathologic findings and genetic analyses, we strongly demonstrate the transplacental metastatic spread from twin 2 to twin 1. In the literature, 9 pairs of concordant twin neuroblastomas, including the current twin, have been presented; and the clinical findings of 5 twin pairs may represent placental metastases from one twin with congenital neuroblastoma to the other twin. This study is the first report presenting the possibility of twin-to-twin metastasis in monozygotic twins with neuroblastoma based on an analysis of the clinical features and genetic aberrations.

Spontaneous resolution of an adrenal mass seen on ultrasound at 17 weeks gestation.

We report a congenital neuroblastoma with findings at 17 weeks gestation that was managed expectantly; this represents the earliest reported finding of a congenital neuroblastoma we could find in the English literature.

Surgical anatomy and embryology of the adrenal glands.

Gross anatomy explains the different surgical approaches to adrenalectomy and the difficulties encountered by surgeons during this procedure. Development of the adrenal glands explains the location of the ectopic sites and excess hormone production by adrenal tumors. The choice of a surgical approach is sometimes difficult and is dependent on (1) the morphology of the body; (2) the volume of the tumor, which necessitates immediate vascular control; and (3) the type of disease, which may necessitate a complete exploration of the abdominal cavity.

Microscopic neuroblastoma in a fetus with a de novo unbalanced translocation 3;10.

We report on a fetus with a de novo unbalanced translocation 3;10 and a microscopic neuroblastoma. The fetus had the karyotypic and phenotypic manifestations of partial dup (3q). The finding of a constitutional chromosomal abnormality and a microscopic neuroblastoma, although possibly coincidental, supports Knudson's two hit hypothesis for development of neuroblastomas and other embryonal tumors. In this case the first mutation is represented by the constitutional abnormality, possibly resulting in the microscopic neuroblastoma. A second mutation affecting the abnormal cells, which may be more prone to mutagenesis, may trigger a neuroblastoma.

Pathology of neuroblastic tumors.

Histogenesis, basic histologic features, nomenclature, and criteria for diagnosis and prognostic classifications based on morphological features of neuroblastic tumors (NTs) are described. NTs that arise from neuroectodermal cells of the adrenal medulla and sympathetic ganglia recapitulate the development of sympathetic ganglion. The following three basic types of NTs are recognized: neuroblastoma (NBs) and ganglioneuroblastomas (GNBs) and ganglioneuromas (GNs). NBs can be undifferentiated, poorly differentiated or differentiating; GNBs have the following three subtypes. (1) nodular, (2) intermixed, and (3) borderline. The conventional and recommended terminology and Shimada terminology of NTs are described (Tables 1 and 2). There are three basic pathologic components of NTs, neuroblastomatous, ganglioneuromatous, and intermediate components (Figs 3, 5, and 7). There are two major prognostic classifications based on morphological features of NTs, Shimada classification and histologic grading (Tables 3 and 4).

Neurocrest and colonic tumors: new clinical syndrome. Report of three cases.

This report describes three patients with both multiple intestinal polyps and tumors of neural crest origin. This combination of findings may represent a new clinical syndrome. The embryologic relationships between tumors derived from endoderm and tumors derived from neurocrest are described. An inherent defect in tissue proliferation or repair is postulated to explain the abnormal growth in these two different cell lines.