Fatal Cardiac Arrest Due to an Unusual Fibroma of the Pulmonary Valve

Fibroma, the second most common tumor in childhood, usually is a large solitary mass involving the ventricular septum or the left and rarely the right free ventricular wall. An unusual case of a 5.2-year-old boy with a large mass originating from the pulmonary valve is reported. The patient presented for evaluation of a murmur. Echocardiography and cardiac magnetic resonance imaging showed a large tumor extending from the distal right ventricular outflow tract through the pulmonary valve and into the main pulmonary artery causing moderate obstruction. The patient had a fatal cardiac arrest while awaiting surgery. The postmortem examination proved the tumor to be a fibroma.     

Small bowel intussusception by local recurrence of an inflammatory myofibroblastic tumor: report of a case and review of the literature

Inflammatory myofibroblastic tumor (IMT) of the ileum is a rare, usually solitary lesion, that frequently presents small-intestinal intussusception and obstruction. We describe an IMT of the ileum in a 4.5-year old child who presented a small bowel intussusception. During laparotomy, an annular mass around the ileum was resected, and the IMT was histologically diagnosed. Three months after the operation, the patients were hospitalized with the symptoms of intestinal obstruction. Laparotomy showed a ileal intussusception. Along the previous suture line of anastomosis, a smooth polypoid tumor was evident. Segmental resection of the ileum, including the tumor mass, was performed. The IMT was immunohistochemically diagnosed. The patient was asymptomatic at 3 year follow-up. A review of the literature for this rare entity emphasizes the importance of immunohistochemical confirmation of its benign nature. Because of the risk of local recurrence, IMT cases should have a long-term follow-up.     

Ten years' experience in the management of cervical teratomas

Five patients with cervical teratoma were seen between 1982 and 1992 in the Maternity and Children's Hospital, Riyadh, Saudi Arabia. All presented with a cervical mass at birth. Their weights ranged between 2.6 and 3.5 kg (average 3.1 kg); all were full-term babies, three girls and two boys. Three cases were diagnosed antenatally; three had a history of maternal hydramnios; three presented with severe respiratory distress at birth and needed endotracheal intubation. All patients underwent surgical treatment except one who died before surgical intervention because of severe respiratory distress. Surgery included complete resection of the tumor. The postoperative courses were uneventful. The histopathology of the tumor showed tissue from all three germ layers. Over 3–11 years of follow-up, none of the patients showed signs of recurrence. Postoperative thyroid function tests and serum alpha-fetoprotein showed no abnormalities. We conclude that antenatal diagnosis help to reduce the morbidity and mortality due to upper airway obstruction. Surgery is safe and represents the only way of treating this tumor.     

Intracardiac yolk sac tumor and dysrhythmia as an etiology of pediatric syncope

This report describes a new etiology of pediatric syncope. Epilepsy, brain anomalies, infection, electrolyte abnormalities, and trauma are commonly identified etiologies of seizures in the pediatric population. We report here a child with third-degree heart block and right ventricular outflow tract obstruction related to an intracardiac tumor presenting with syncope and seizure-like activity. Echocardiography revealed a large (3 x 8-cm) intracardiac mass filling the right atrium, extending across the tricuspid valve into the right ventricle and crossing the atrial septum into the left atrium, extending into the left ventricular outflow tract. She underwent emergent cardiopulmonary bypass with removal of the majority of the tumor mass, clearing both the left and right ventricular outflow tracts of obstruction and repairing the tricuspid valve. Postoperative cardiac conduction remained blocked and required permanent pacing. The initial serum alpha-fetoprotein level was grossly elevated, and the tumor showed characteristic histopathologic features of a yolk sac tumor. Four years after the completion of her chemotherapy, she remains clinically well, with no evidence of recurrent tumor by echocardiography or radiographic studies, and her alpha-fetoprotein remains in the normal range. The clinical manifestations of tumor infiltration of the heart with complete heart block resulting in loss of consciousness with tonic-clonic movements are detailed. Although rare, cardiac syncope has multiple known causes and should be suspected in any patient with sudden loss of consciousness and pallor. In the pediatric population, cardiac rhythm disturbances are typically the result, rather than the cause, of acute cardiac emergencies. Pediatricians should be aware of depressed cardiac output and dysrhythmias as etiologies of new-onset syncope. Evaluation should include a cardiac assessment with electrocardiogram to exclude a life-threatening arrhythmia as a potential cause.     

Right Atrial Primary Cardiac Osteosarcoma

A previously healthy 14-year-old boy with an atypically located primary cardiac osteosarcoma filling the right atrium with significant extension into both systemic and pulmonary venous structures presented with a clinical picture of heart failure. Imaging showed a large right atrial mass with posterior extension into right pulmonary veins and inferior extension into the inferior vena cava causing near total obstruction of systemic venous return to the heart. Bone marrow biopsy showed no evidence of marrow involvement by metastatic malignancy. Histopathology was a high-grade osteosarcoma. Partial tumor debulking was achieved via right atriotomy.     

A case of incomplete Currarino triad with malignant transformation

A case of an incomplete Currarino triad is reported. The baby underwent an emergency laparotomy due to a life-threatening intestinal obstruction caused by severe rectal stenosis. During the posterosagittal anorectoplasty (PSARP), a presacral teratoma was identified and resected. The tumor recurred three times; she initially responded to chemotherapy, but nonetheless died at the age of 4 years. In cases with evidence of anorectal stenosis, a presacral mass should be suspected. PSARP is the best choice of treatment for both the anorectal anomaly and excision of the presacral mass. The presacral region should be followed up closely for recurrence of the tumor. Accepted: 13 July 1998     

Colon carcinoma treated with oxaliplatin and capecitabine in a 12-year-old child

Background

XELOX (oxaliplatin 130mg/m² iv, capecitabine 1000mg/m² bid oral d1–14, q3w) chemotherapy has never been used in children. In this report, we present a case of a 12-year-old girl with colon adenocarcinoma, treated with surgery and XELOX chemotherapy.

Methods

On admission, the girl complained of abdominal pain and intestinal obstruction. Physical examination revealed a distended abdomen with tenderness on the left upper quadrant. Barium enema revealed a stenotic lesion at the distal end of the transverse colon, and abdominal computed tomography showed acute obstruction and a colonic mass. Laparotomy was performed after the failure of conservative treatment.

Results

The mass was originated from the transverse colon. Frozen sections of the specimens revealed an adenocarcinoma. Transverse colectomy was performed and regional lymph nodes were removed. Pathological examination confirmed that the mass was a poorly differentiated adenocarcinoma, and XELOX chemotherapy was used. No evidence of recurrent or metastatic tumor was found after 18 months.

Conclusion

Although complete resection is the most effective treatment, XELOX chemotherapy is beneficial to the improvement of clinical outcome of patients with colon adenocarcinoma.     

Nasal chondromesenchymal hamartoma: radiographic and histopathologic analysis of a rare pediatric tumor

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign pediatric tumor that was described in 1998. Only 19 cases are reported in the literature. We present a 15-year-old girl with nasal obstruction and recurrent sinusitis. Her medical history was significant for a rare ovarian tumor and pleuropulmonary blastoma. CT demonstrated a partially calcified soft-tissue mass obstructing the nasal cavity. The patient underwent endoscopic surgical excision. Histologic and immunohistochemical analyses of the tumor were consistent with NCMH. The imaging characteristics of the tumor are reviewed. NCMH may be part of a syndrome associated with other pediatric neoplastic and dysplastic disease.     

Transient obstruction of the kidney and hypertension due to neonatal adrenal hemorrhage. Case report

A case of neonatal adrenal hemorrhage associated with transient obstruction of the kidney and hypertension is reported. Sonography demonstrated a mass in the right suprarenal area, consistent with hemorrhage into the adrenal gland. DTPA renal scan showed prolonged retention of the injected material in the right kidney, consistent with obstructed outflow from the renal collecting system. Gradual decrease in the size of the suprarenal mass was associated with relief of the renal obstruction as evidenced by a normal repeat renal scan, and a return to normal of the blood pressure. It appears that the cause for the hypertension was the acute renal obstruction, which was due to either direct pressure on the proximal ureter by a large adrenal mass or secondary to displacement of the kidney by the mass resulting in kinking of the proximal ureter.     

Functional outcome after pyeloplasty for unilateral symptomatic hydronephrosis

The functional outcome and factors influencing improvement after pyeloplasty for ureteropelvic junction (UPJ) obstruction are still debated. This retrospective study was aimed at evaluating the factors associated with functional improvement in symptomatic unilateral hydronephrosis (HDN). Patients (n = 68) who underwent successful pyeloplasty for unilateral symptomatic UPJ obstruction without any other associated urological abnormality were included. Preoperative evaluation included a diuretic renogram (DR) to confirm obstruction and assess the split renal function (SRF). A follow-up DR was obtained 3 months and 1, 2, and 5 years postoperatively. An absolute increase in the differential function of the operated kidney by over 5% was considered significant; such kidneys were classified as improved (group A) and the others as unimproved (group B). The difference between the preoperative and 3-month SRF was highly significant (P < 0.001). Significant (P < 0.01) improvement in SRF continued until 1 year after pyeloplasty. Patients who presented with a mass had significantly greater improvement (P < 0.05) than those who presented with other clinical features. In group A, a significantly higher number of patients presented with a mass. It was also evident that patients aged less than 1 year showed significantly greater (P < 0.01) improvement in SRF than older patients. Thus, in unilateral symptomatic HDN with impaired function, patients who present with a mass or those in whom pyeloplasty was performed before 1 year of age showed significantly greater improvement.     

Acquired pulmonary stenosis: Ultrasonographic diagnosis

A 15-year-old boy presented with chest pain and a new heart murmur. The clinical diagnosis of pulmonary stenosis was confirmed by two-dimensional and Doppler echocardiographic examinations. A large, encapsulated, partly solid and partly cystic tumor in the anterior mediastinum, visualized by ultrasonography, was compressing the main pulmonary artery and producing the right ventricular outflow tract obstruction. The tumor was removed surgically and was found to be a benign teratoma. Postoperative ultrasound examination of the right ventricular outflow tract showed no evidence of obstruction. We conclude that: (1) there are subtle clinical diagnostic differences between intrinsic and extrinsic pulmonary stenosis; (2) ultrasound examination can make cardiac catheterization unnecessary; and (3) relief of the extrinsic cause of pulmonary stenosis should be curative.     

Lymphangiomum cysticum congenitum of the left half of the face in a newborn infant

In a mature dystrophic newborn a huge tumor of the face was evident. The left eye could not be seen, the left choana was obstructed, the left angle of the mouth was pushed caudally. Sonographically cystic and solid areas could be found. The cranial computer tomography showed normal intracerebral structures but some evidence of infiltration into the frontal brain. The left ocula was widened and the left eye was found within the tumor mass. Angiographically an abnormal vascularisation of the tumor could not be seen. In spite of corticoid therapy a rapid growth led to life-threatening obstruction of the airways. At the age of 6 weeks the tumor was totally extirpated. A small piece of dura mater was replaced by periost. The wound could not be closed. Histologically the diagnosis of lymphangioma cysticum congenitum was made and increased growth tendency were found. Four weeks postoperatively the baby was discharged. Several cosmetic operations with unpredictable results will be necessary. A close psychologic guidance is recommended. Localisation, size, and life-threatening growth of a benign lymphangiomum are very uncommon.     

Hämatometrokolpos

Case reports. We report on 3 girls aged 12 to 15 years with a history of recurrent backache as well as acute lower abdominal pain. In all three adolescents sonography revealed bilateral pyelectasis as well as dilated ureters. One girl additionally had acute urinary retention. In all 3 girls a huge retrovesical cystic tumor could be shown, which had caused obstruction of the urinary tract. The uterus was located at top of the cystic lower abdominal mass. In two patients the cavum of the uterus was dilated. Clinical examination revealed an intralabial protruding mass caused by an imperforate hymen. After resection of the hymenal plate prompt recovery of urinary retention and pyelonectasy occurred.     

An unusual cause of meconium peritonitis in a foetus

Abstract:   Meconium peritonitis is a sterile chemical peritonitis resulting from intestinal perforation in-utero. We present a preterm male neonate weighing 1820 g in whom foetal meconium peritonitis was diagnosed by antenatal utrasonogram. Examination of the newborn in the immediate postnatal period revealed a firm lump of size 5 × 3 cm in the right lumbar and umbilical area. Rest of the abdomen was normal without any clinical evidence of intestinal obstruction. X-ray of abdomen done at 3 hours of life showed a cystic mass with calcified margin. Ultrasonography of abdomen ruled out any definite cause of intestinal obstruction and perforation. He was kept on conservative management. At 11 hours of life the neonate passed a small sticky white mucus plug per rectum followed by black colored meconium of normal consistency 2 hours later. The bowel habit remained regular thereafter. Screening for intrauterine infections and cystic fibrosis was negative. He is under regular follow-up and is now 6 months old with normal bowel habit. A lump of size 2 × 2 cm is still palpable and a small calcified mass is persistent in x-ray. Role of an intestinal mucus plug as the potential factor for intestinal obstruction and subsequent perforation has been highlighted in the present case.     

Primary cardiac leiomyoma of the ventricular septum: a rare form of pediatric intracardiac tumor

A case of ventricular septal tumor diagnosed in 7-month-old boy is reported. An echocardiogram was performed for investigation of a heart murmur in an otherwise healthy infant. He remained asymptomatic, and the tumor had a very slow growth. When the boy was 9 years old, partial surgical excision was performed for right ventricular outflow tract obstruction. Histology showed a primary cardiac leiomyoma. To the authors’ knowledge, a primary cardiac leiomyoma of the ventricular septum has not been reported previously.     

MEGADOSE METHYLPREDNISOLONE FOR EVANS SYNDROME

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

Recurrent pneumonia revealing a bronchial carcinoid tumor: report of two cases]

Carcinoid tumors are the most common endobronchial tumor in the pediatric population, and represent a rare cause of airway obstruction. The authors report two cases of boys aged 10 and 11 years old, who presented with a 12-month history of recurrent pneumonia. Bronchial endoscopy showed an endobronchial tumor. Chest CT-scan identified local extension and lung-associated lesions; octreoscan was performed to detect distant metastases. Histopathological study concluded in typical carcinoid tumor. The outcome after surgical conservative resection is uneventful with a follow-up of 7 and 26 months. Bronchial tumors must be considered in children with recurrent pneumonia or persistant respiratory symptoms, and require CT scan and bronchial endoscopy for their diagnosis.     

Combined chemotherapy and tracheobronchial stenting for life-threatening airway obstruction in a child with endobronchial non-Hodgkin lymphoma

Endobronchial involvement in non-Hodgkin lymphoma is rare even in the presence of advanced disease. A 15-year-old boy presented with progressively worsening dyspnea with occasional hemoptysis for 1 week prior to admission. Three days later, he was intubated due severe dyspnea with complete atelectasis of the right lung. Fiberoptic bronchoscopy disclosed an endobronchial mass almost occupying the right main bronchus. He underwent partial resection of the endobronchial tumor with rigid bronchoscopy. An airway stenting was used in this patient because he had severe tracheal obstruction from the tumor. The compromised airway was alleviated by combined chemotherapy and tracheobronchial stenting.     

Abdominal Inflammatory Myofibroblastic Tumor: Report on Four Cases and Review of Literature

Background

The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT.

Cases Presentation

We herein present four cases of AIMT in different ages (range: 3.5 to 13 years) and in different organs (stomach, periduodenal, mesenteric, and colon). There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years.

Conclusion

As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up.     

Unilateral nasal obstruction in children: Pai syndrome

Unilateral obstruction of the nasal cavity in children is mainly caused by the introduction of foreign bodies further stated with rhinorrhea and fetid odor. Less commonly, it can be traumatic, neoplastic, due to congenital malformation or iatrogenic. Symptoms of congenital intranasal mass may present at birth, or go unnoticed and be a finding in a routine pediatric examination. Patient evaluation should include imaging studies to guide the diagnosis and rule out intracranial extension. A syndrome associated with congenital nasal tumor should be suspected when other abnormalities are present. Pai syndrome is a rare genetic disorder. Its manifestations are craniofacial being congenital nasal polyp his main marker. We present a patient with unilateral nasal respiratory failure secondary to congenital nasal lipoma, with craniofacial anomalies belonging to Pai syndrome. Nasal obstruction was successfully surgically resolved.