Benign lipoblastoma arising in the neck

Lipoblastoma and lipoblastomatosis are rare benign tumours of embryonal fat that exhibit a tendency to invade locally but not to metastasise. This condition most often presents before the age of 3 years, affects males more than females, and is typified by a slowly-growing, usually subcutaneous mass arising in a limb. Cases involving the head and neck are extremely rare, with only nine cases in the neck having been described to date. This tenth case arose in a 13-month-old male presenting with a rapidly enlarging lump in the right side of the neck, clinically mimicking a cystic hygroma. Diagnosis is by histology, and treatment involves complete surgical resection. We report this tenth known case of lipoblastoma in the neck and review the literature. Accepted: 4 February 1997     

Coexistence of an adrenocortical carcinoma with an abdominal ganglioneuroma in a child

We report a 3-year, 5-month-old boy with an adrenocortical carcinoma. These tumours are rare and highly malignant in childhood. In most cases they are functional, secreting adrenocortical hormones. In this case there was a misleading coexistence with a second abdominal neoplasm, which was a ganglioneuroma; this is a rare benign tumour arising from the sympathetic nervous system. The imaging investigations and their findings are discussed and correlated with pathology. Received: 15 January 1997 Accepted: 15 October 1997     

Jaw and gum tumours in children

Introduction  

Jaw and gum tumours are rare conditions in children. Children presenting with jaw tumours during 2001 till 2007 are presented. Their modes of presentation, operative procedures and findings, histology and post operative results have been described.     

Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children

Rhabdomyosarcoma is a soft-tissue malignancy that represents approximately 4–8% of all solid tumours in children and commonly arises from the head and neck and genitourinary system. Intraperitoneal rhabdomyosarcoma, in particular with omental involvement, has been rarely reported in the literature. Furthermore, reports of omental rhabdomyosarcoma of embryonal origin do not exist, to our knowledge. We report two cases of omental embryonal rhabdomyosarcoma affecting children and illustrate the imaging characteristics of this rare tumour.     

Cervical paraganglioma--a case report and review of all cases reported to the Manchester Children's Tumour Registry 1954-2004

We report a 6-year-old male with left-sided ptosis, aniscoria and an initially missed slow growing left-sided neck mass, which was surgically excised when he was 9 years old and confirmed to be a paraganglioma. Seven years later he developed recurrent symptoms and was found to have a recurrence in the anterior mediastinum. We also report on all cases of cervical paragangliomas registered with the Manchester Children's Tumour Registry (MCTR) for the 50-year period 1954-2004. Paragangliomas are very rare tumours in the head and neck but should be considered in the differential diagnosis of neck masses especially when presenting with Horner syndrome. Recurrent symptoms and signs of hypertension herald recurrence. As these tumours can form part of a familial syndrome, long-term follow-up is necessary. Family members should be screened for early detection.     

Melanotic neuroectodermal tumour of infancy: CT and MR findings

Background

Melanotic neuroectodermal tumour of infancy (MNTI) is a rare neoplasm of neural crest origin.

Objective

To describe three further cases of MNTI, with emphasis on CT and MRI findings.

Materials and methods

Data for children with histologically confirmed MNTI following biopsy or surgery were retrieved. Three children with available imaging at the time of diagnosis were included in the study.

Results

All three children had primary tumour in the head and neck region: one in the maxilla, one in the occipital bone (extra-axial but with intracranial extension) and one with an unusual tumour growing exophytically from the subcutaneous tissues adjacent to the occipital bone. All tumours were iso/hypointense both on T1- and T2-weighted MRI, and showed marked contrast enhancement in their non-ossified components. CT allowed identification of bone destruction and remodelling.

Conclusion

Our findings are consistent with previously reported cases of MNTI regarding age at presentation and location in the head and neck region. Our MR findings did not demonstrate the typical pattern of T1-shortening expected from melanin deposition.     

Neonatal tumours in Malaysia: a call for heightened awareness

Purpose  

Neonatal neoplasms are rare tumours notorious for their atypical presentation and unpredictable behaviour. Their optimal treatment remains uncertain, a dilemma compounded by the deleterious effects of adjuvant chemo- or radiotherapy during this vulnerable period of growth. This paper examined the relatively high incidence of these tumours and its impact on paediatric surgery in Malaysia.     

Spindle cell lesions of the head and neck mimicking rhabdomyosarcoma in children

Malignancies of head and neck are uncommon among children. The most commond solid tumors to occur in this region are rhabdomyosarcoma, Hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to hav e benign spindle cell lesions. These rare lesions should bepart of the differential of children with a head and neck mass.     

Gonadal tumours in children]

Gonadal tumours are rare in children. Benign tumours are more frequent in girls than in boys (3 cases among 4 cases in girls and 1 case among 2 or 3 cases in boys), due to the prevalence of functional ovarian pathology (functional cysts, ovarian torsion). Whatever the clinical presentation of these tumours (mainly abdominal pain or mass in girls, scrotal mass in boys) and even before an emergency procedure, radiological investigations (abdominal x-rays, ultrasound scans) and biological ones (alpha-foeto-protein and human chorionic gonadotropin dosages) should be performed in order to precise the diagnosis. In benign tumours (mature teratoma, cystadenoma), a conservative surgical procedure (tumorectomy with ovarian preservation) should be attempted. Malignant or potentially malignant tumours (malignant germ cell tumours, stromal tumours) have an excellent prognosis, only if they are treated with a close collaboration between surgeons and oncologists.     

Congenital tumours involving the head, neck and central nervous system

Congenital intracranial tumours are uncommon and differ from those occurring in older children in clinical presentation, imaging characteristics and prognosis. These tumours are often detected incidentally on routine prenatal US and/or fetal MRI. Hence, the paediatric radiologist should be familiar with the features of those lesions that should be included in the differential diagnosis. In general, the prognosis of these conditions is poor owing to large tumour size and the limitations of adjuvant therapy at such a young age. Congenital lesions involving the head and neck region require a meticulous imaging approach using both US and MRI techniques to better guide prenatal planning and fetal or neonatal surgical procedures.     

Sialoblastoma and hepatoblastoma in a newborn infant

We report a case of a newborn infant who had simultaneous sialoblastoma and hepatoblastoma tumours at birth. The diagnoses were made on post mortem examination. Both of these are rare tumours in the neonatal period. Pediatr Blood Cancer 2009;52:883–885. © 2009 Wiley‐Liss, Inc.     

Transitional cell papilloma of the bladder in a child: a case report and review of literature

Transitional cell papillomas of the bladder are tumours of epithelial origin that are extremely rare in children. A large number have been found to be sited adjacent to one of the ureteric orifices. They are benign and endoscopic excision has been considered curative; however, the rate of recurrence reported in the literature is fairly high, with the earliest recurrence being at 3 months. We report the case of a 7-year-old girl who presented with the rare lesion situated just above the right ureteric orifice.     

Spontaneous rupture of a malignant rhabdoid tumour of the liver

Malignant rhabdoid tumours (MRT) of the liver are rare. The criteria for pathological diagnosis are clearly defined, but the clinical behaviour of these tumours is still emerging. We report a MRT of the liver with the rare clinical presentation of spontaneous rupture. Accepted: 23 september 1997     

Successful management of a retroperitoneal kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using alpha-interferon.

It has been shown recently that Kasabach-Merritt phenomenon, the association of a vascular tumour and consumption coagulopathy, does not--as previously thought--complicate "classical" infantile hemangiomas but distinctive entities called kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), both tumours on the same neoplastic spectrum. These tumours have been found in the neck, face, thorax, abdomen, retroperitoneum and limbs and are associated with a mortality rate of as high as 30 %. Several therapeutic modalities, including alpha-interferon, vincristine, radiotherapy and surgery have been reported in the literature. We report a case of retroperitoneal kaposiform hemangioendothelioma regression using alpha-interferon and discuss the current knowledge of this entity and its treatment.     

Tumores germinales intracraneales: revisión de 21 años

Introduction

Intracranial germ cell tumours are rare in children. They are a heterogeneous group of neoplasms that show different clinical manifestations despite having a common origin.

Rare malignant mid-femoral tumours in the first decade of life

The most common femoral shaft tumours in children are eosinophilic granuloma and Ewing's sarcoma. Three children in the first decade of life with rare femoral shaft tumours are reported. There was one osteosarcoma and two chondrosarcomas. Radiographic differential diagnosis of the femoral shaft tumours and microscopic diagnostic difficulties of chondrosarcoma are discussed.     

Fetal and neonatal liver tumours

Liver tumours are rare in children and account for about 5% of all tumours in the fetus and newborn. The most frequently occurring are benign vascular tumours and mesenchymal hamartomas although malignancy in the form of hepatoblastoma is a possibility. While the diagnosis can be suspected antenatally (by ultrasound and MR scan), a precise diagnosis is often difficult due to the complexity of the tumours. Inutero development of such tumours may be associated with polyhydramnios, fetal hydrops and extreme cases the maternal mirror syndrome. Postnatal symptoms may include abdominal distension, cardiac failure, consumptive coagulopathy and bleeding due to tumour rupture, but is dependent on the nature of the actual tumour. Treatment options may include watchful waiting, surgical resection, hepatic artery embolisation/ligation and chemotherapy.     

A pictorial review of imaging of abdominal tumours in adolescence

Neoplastic abdominal tumours, particularly those originating from embryonal tissue (such as hepatoblastoma and nephroblastoma) and neural crest cells (such as neuroblastoma), are well-documented in young children. Neoplasms of adulthood, most commonly carcinoma of different visceral organs, are also well-documented. Abdominal tumours in adolescence constitute a distinct pathological group. The radiological features of some of these tumours have been described only in isolated reports. The purpose of this pictorial essay was to review the imaging findings of various kinds of abdominal tumours in adolescent patients (with an age range of 10–16 years) who presented to the Children Cancer Center of our institution in the past 15 years. Some tumours, though rare, have characteristic imaging appearances (especially in CT) that enable an accurate diagnosis before definite histological confirmation.     

Head and neck teratomas in children

A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone post-operatively. Head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority. Accepted: 8 November 1999     

Fetal rhabdomyoma of the lower extremity

We report a rare case of cellular fetal rhabdomyoma in a 9‐year‐old male, in the unusual location of right lower thigh. These tumors are more common in the head and neck region; and this case the second such case to be reported in the thigh. Fetal rhabdomyoma is a benign tumor of the skeletal muscle, showing varying degrees of skeletal muscle maturation. The present report discusses the histopathological features, the differential diagnosis and the importance of making the correct diagnosis for proper management of this rare entity. Pediatr Blood Cancer 2009;52:881–883. © 2009 Wiley‐Liss, Inc.