Management of cervicomediastinal neuroblastoma presenting with life-threatening tracheal obstruction in infancy.

The authors report on 3 infants below 6 months of age at diagnosis, with cervicomediastinal neuroblastoma who presented with life-threatening tracheal obstruction as an oncologic emergency. These neuroblastomas were characterized by favorable biology and chemoresistance. All initially grew rapidly before spontaneously regressing. Nerve injuries occurred in all patients as a result of tumor location. Maintenance of the airway until the expected spontaneous regression was a critical component in the management of these patients.

Fetal mediastinal tumor of neuroepithelial origin in a case of missed abortion.

We report a rare case of a primitive embryonal tumor discovered in the upper anterior mediastinum during routine autopsy of a macerated fetus at the 18th week of gestation. Our diagnosis was based on autopsy findings and histologic examination, which showed neuroepithelial differentiation of the tumor with frequent ependymal-type rosette formation; no structures of other germ cell layer origin were revealed. Additional positive immunohistochemical staining for CD56, CD57, and neurofilament protein confirmed the neural origin of the tumor, whereas the genetic analysis showed no MYCN gene amplification and no 11q23 deletion or rearrangement of EWS locus (22q12). Our findings exclude the possibility of teratoma and support the diagnosis of undifferentiated primitive tumor of neuroepithelial origin uncommonly located in the anterior mediastinum at the early 2nd trimester of gestation in a case of missed abortion.

Bronchogenic cyst associated with pericardial defect: case report and review of the literature.

Partial defect of the pericardium combined with bronchogenic cyst is a very rare congenital anomaly. We describe the case of a 32-year-old man with a partial defect of the left pericardium and a bronchogenic cyst arising from the border of the pericardial defect. The cyst was successfully resected with the harmonic scalpel by three-port videothoracoscopic approach.

Mediastinal and neck kaposiform hemangioendothelioma: report of three cases.

Kaposiform hemangioendothelioma is an aggressive vascular tumor, named for its striking histologic resemblance to Kaposi sarcoma and locally invasive growth. Mortality is high, and ranges from 10% to 24% for all kaposiform hemangioendothelioma lesions, with a significantly higher mortality for deep soft-tissue or visceral lesions occurring in infants less than 6 months. Mediastinal and neck kaposiform hemangioendothelioma in particular merit special discussion, as involvement of these critical anatomic locations results in significant site-specific therapeutic challenges due to invasion of vital structures, inherent delays in establishing histopathologic confirmation, and difficulties in monitoring disease status. We report our experience with three cases of mediastinal and neck kaposiform hemangioendothelioma, emphasizing the unique diagnostic and management challenges, variable response to treatment and outcome of this anatomic variant of kaposiform hemangioendothelioma.

Fetal mediastinal lymphangiomas.

OBJECTIVE: The purpose of this series was to evaluate the prenatal sonographic findings and postnatal outcomes in 2 fetuses with mediastinal lymphangiomas. METHODS: The fetal images were compared with postnatal imaging and surgical findings. RESULTS: The 2 fetuses had anechoic mediastinal masses at 25 and 22 weeks, which proved to be lymphangiomas. One, located in the anterior mediastinum, also enveloped the superior vena cava, brachial plexus, phrenic nerve, larynx, and lower parts of the neck vessels and extended into the subcutaneous tissues of the anterior chest wall through an intercostal space. In the second patient, the lymphangioma appeared to be a unilocular cyst, which involved the deep tissues of the neck as well as the posterior and lateral mediastinum. Both required 2 interventions after birth. CONCLUSIONS: Fetal mediastinal lymphangiomas appeared anechoic and sent extensions into the neck in the first case, around the superior vena cava, through the intercostal spaces to the skin, and around the brachial plexus in the second case, and deviated the trachea in both cases. In 1 case, there was also ectasia of the superior vena cava. This ability to entwine around vital structures can make it difficult to determine the extent of involvement on antenatal sonography and to remove lymphangiomas completely, and recurrence is common.

Teratomas of the neck and mediastinum in children.

This retrospective study reviews a series of teratomas of the neck and mediastinum aiming at defining the features of these particular locations. We recorded prenatal diagnosis, perinatal management, clinical and radiologic features, pathology, surgical strategies and results in cervical and mediastinal teratomas treated over the last 10 years. During this period we treated 66 children with teratoma of which 11 (6 male and 5 female) had cervicomediastinal locations. Five babies had cervical teratomas extended into the anterior mediastinum in two cases. Prenatal diagnosis was made in three (two with polyhydramnios). Four babies were born by C-section and only one had a successful EXIT procedure. The diagnosis was confirmed by imaging and increased AFP. Surgical treatment involved total tumor removal and in one case subsequent removal of lymph node metastases. All children survived except one in whom airway could not be cleared at birth. Two children bear mild hypothyroidism. During the same period six patients aged 0-17 years were treated for mediastinal teratoma. Only one was prenatally diagnosed and only two had some dyspnea. Removal was performed either by median sternotomy, thoracotomy, or thoracoscopy. They all survive and are free of disease. Teratomas of the neck may cause fetal disease and unmanageable neonatal airway obstruction. Prenatal diagnosis and planned multidisciplinary management are mandatory at birth. In contrast, only some mediastinal tumors cause respiratory embarrassment. Although benign, these tumors are sometimes immature and may metastasize to regional lymph nodes. Total surgical removal is curative. Thyroid insufficiency may be present at birth in cervical teratomas and may be aggravated by surgery.

Congenital giant aneurysm of the left innominate vein: is surgical treatment required?

Congenital aneurysms of the thoracic venous system are rare. In particular, innominate venous aneurysms are extremely rare. We describe a 16-year-old girl whose chest x-ray suggested a mediastinal tumor. Three-dimensional contrast-enhancement magnetic resonance venography showed a giant sacciform aneurysm of the left innominate vein and dilatation of the right innominate vein. The patient was asymptomatic, and there were no significant physical findings. Therefore, the patient is being followed without surgical treatment.

Congenital mediastinal immature teratoma: a case report with autopsy findings.

A full-term newborn with karyotype 46, XX was delivered by cesarean section. She had severe respiratory distress and substernal retraction, and underwent emergency operation, but she died on the same day due to respiratory failure. The mother, 26-year-old prima gravida with no history of twinning, had been examined with ultrasonography at the 34th week of her pregnancy, which revealed a fetus with edema of head and neck region, a probable diaphragmatic hernia, polyhydramnios, and a large mediastinal mass with solid and multicystic parts with hypoplasia of the lungs. Autopsy revealed a 9 x 5 x 3 cm lobulated mediastinal mass with both solid and cystic areas, displacing the lungs and the heart postero-inferiorly and thymus anteriorly. The lungs were hypoplasic. Microscopically, the mass showed mature epithelial and mesenchymal tissues with primitive mesenchyme and immature neuroepithelium. All these findings led to the diagnosis of an immature teratoma. Mediastinal teratomas are rare and life-threatening, but early diagnosis and surgical intervention in a newborn with sufficient lung maturation may provide a long survival.

[Mediastinal teratomas in children]. / Mediastinální teratomy u detí.

The objective of the work is a clinical and histopathological retrospective study of rare mediastinal teratomas in children. The authors present their experience with the treatment of eight children within the range from neonates to the age of 15 years with a histologically confirmed diagnosis of mediastinal teratoma. In two neonates the first symptom was postnatal dypnoea, teratomas in older children were diagnosed along with symptoms of acute respiratory infection. Complete resection of the tumor was performed in all patients, in neonates emergency operations were involved. Four tumors were histologically classified as mature teratomas, three as differentiated teratomas with an immature component and one as a malignant teratoma. The malignant teratoma contained mature tissues as well as tissues of embryonic carcinoma and a yolk sac tumor. Seven children with a mature or differentiated teratoma are without signs of disease 5-15 years after surgery. During the follow-up of the 15-year-old boy with the malignant teratoma the elevated alpha-fetoprotein serum level was evidence of a relapse of the disease after termination of comprehensive treatment. From the results ensues that radical extirpation is sufficient treatment for mature and differentiated teratomas. Malignant teratomas are indicated for primary resection and postoperative chemotherapy. Long-term follow up of alpha-fetoprotein and human beta-choriogonadotropin levels, clinical controls and controls by imaging methods are indicated in children with an immature or malignant component of the tumor.

[Cystic lymphangioma of the neck and mediastinum: are there acquired forms? Report of 37 cases]. / Les lymphangiomes kystiques du cou et du médiastin: existe-t-il des formes acquises? A propos de 37 cas.

BACKGROUND: In 9 out of 10 cases, lymphangiomas are observed during the first years of life, generally located in the neck. Rare lymphangiomas have been reported in adults, usually in an intrathoracic localization, raising the hypothesis of an acquired origin. PATIENTS AND METHODS: A retrospective analysis of patients undergoing surgery for lymphanioma of the neck or mediastinum was conducted. Pathology reports, clinical data and medical imaging (CT) were reviewed and operative and post-operative findings were analyzed in order to determine whether the cases could be divided into congenital and non-congenital forms. RESULTS: Data were obtained on 37 patients (23 men and 14 women, mean age 45 years, range 8-77). Four lesions (11%) were located in the neck: 3 in children and one in an elderly subject (77 yrs). Thirty-three tumors (89%) were located in the mediastinum. In 5 cases, the tumor was located in the phrenic nodes of the anterior mediastinum. These lymphangiomas occurred in young adults, had a CT density less than liquid with enhancement after contrast injection, and had a malformative vascular component proven anatomically and histologically (hemolymphangioma). These elements favored a congenital orgin. In 28 cases (76.6%) the tumor involved nodes in the posterior or middle mediastinum. They occurred in older adults and were purely liquid with no vascular component. These lymphangiomas were undoubtedly acquired. CONCLUSION: In children, adolescents and young adults, lymphagiomas are found in the neck or anterior mediastinum and have a tissular component. These tumors should be considered as congenital lymphangiomas. In older adults, lymphangiomas are found in the posterior or middle mediastinum and are purely liquid cysts suggesting an acquired origin.

Mediastinal teratoma in a neonate.

A neonate with severe respiratory distress due to a benign mediastinal teratoma (MT) is reported. Despite early and easy surgical excision of the tumor, the child died due to poor cardiac function. Only ten cases of MT in neonates have been reported in the literature so far. While the tumor has been known to interfere with lung development in utero, postnatal myocardial dysfunction due to poor heart development has not been previously documented.

Congenital mediastinal dumbbell neuroblastoma with spontaneous regression of liver metastases.

We report a patient with a congenital mediastinal dumbbell neuroblastoma (NB) presenting as respiratory distress at birth rather than a neurologic deficit. The tumor did not respond to low-dose chemotherapy, but radiotherapy and surgery were effective. The patient was observed postoperatively without further therapeutic intervention considering the favorable biologic factors despite the appearance of liver metastases, which ultimately regressed spontaneously. The authors believe that most congenital NBs have favorable outcomes with a possibility of spontaneous regression, and that an understanding of the biologic features of tumors is important to avoid overtreatment of lesions with a favorable prognosis. Keywords Congenital neuroblastoma. Dumbbell tumor. Liver metastasis. Spontaneous regression

The complete spectrum of neurocristopathy in an infant with congenital hypoventilation, Hirschsprung's disease, and neuroblastoma.

Neuroblastoma, Hirschsprung's disease, and central hypoventilation (Ondine's curse) are considered aberrations of neural crest cell growth, migration, or differentiation, and as such are considered to be under the general heading of neurocristopathy. Their combined occurrence in a newborn infant presenting with total colonic aganglionosis, central hypoventilation, and multifocal neuroblastoma had not been reported previously. A 2.3-kg white full-term girl required endotracheal intubation because of persistent apnea in the first hours of life. She had progressive abdominal distension and failure to pass meconium; a barium enema was performed, which showed microcolon with meconium pellets at the distal ileum. During laparotomy the distal ileum was found to be obstructed with inspissated meconium; an ileostomy and appendectomy were performed. The resected specimens were aganglionic. An additional 20 cm of aganglionic ileum was removed, and a normally innervated ileostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an extensive apnea workup, including electroencephalogram, magnetic resonance imaging (MRI), bronchoscopy, and pH probe study, were normal. Sleep studies showed congenital central hypoventilation syndrome, and the patient underwent a tracheostomy. At 3 months, an abdominal ultrasound examination performed within a septic workup showed a right suprarenal mass extending across the midline. Thoracic and abdominal MRI scans showed large bilateral adrenal and posterior mediastinal masses. The serum catecholamines and ferritin level were markedly elevated, suggestive of neuroblastoma. In light of the child's multiple problems, the family chose to forgo further workup (including a tissue biopsy) and therapy. In the following 2 months her tumor load rapidly progressed, and she died of respiratory insufficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Intrathoracic congenital fibrosarcoma. A case report.

Congenital fibrosarcoma is a rare tumor that usually presents as a mass lesion involving the arm or leg of a neonate. No ultrasonographic description of such a neoplasm in the prenatal period has been reported. We present the sonographic findings of a tumor, discovered in an unusual site in utero, that, on postmortem examination, was demonstrated to be a fibrosarcoma.

Congenital posterior mediastinal teratoma--a case report.

Almost all mediastinal teratomas are found in the anterior mediastinum. We describe a case of a 41-day-old baby girl who had teratoma in the posterior mediastinum. She presented at the age of 6 days with the problem of progressive jaundice and was found to have septicaemia. During septic screening, chest X-ray revealed a mass in the right mediastinum. Ultrasound and CT-scan of the chest showed a multiloculated mass at the right posterior mediastinum. Fine needle aspiration biopsy of the mass confirmed a teratoma.