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Infants with hypertrophic pyloric stenosis are usually diagnosed at about 3 to 8 weeks of age. The clinical onset of symptoms in preterm babies is observed normally at a later age than in term or post-term newborns. This report describes a rare case of a 2-day old preterm twin girl presenting with drinking laziness and recurrent vomiting. Five days after the beginning of symptoms and after several studies, including an upper gastrointestinal contrast study, the diagnosis of hypertrophic pyloric stenosis was made and confirmed at surgery. The postoperative course was uneventful. Interestingly, the mother of the child herself had a history of postnatal surgery on her fifth day of life due to congenital hypertrophic pyloric stenosis. To our best knowledge, this is the first report in the literature describing congenital hypertrophic pyloric stenosis in a mother and her child.
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Introduction: Ewing sarcomas of the chest wall, historically known as "Askin tumors" represent highly aggressive pediatric malignancies with a reported 5-year survival ranging only between 40 and 60% in most studies. Multimodal oncological treatment according to specific Ewing sarcoma protocols and radical "en-bloc" resection with simultaneous chest wall repair are key factors for long-term survival. However, the surgical complexity depends on tumor location and volume and potential infiltrations into lung, pericardium, diaphragm, esophagus, spine and major vessels. Thus, the question arises, which surgical specialties should join their comprehensive skills when approaching a child with Ewing sarcoma of the chest wall. Patients and Methods: All pediatric patients with Ewing sarcomas of the chest wall treated between 1990 and 2020 were analyzed focusing on complete resection, chest wall reconstruction, surgical complications according to Clavien-Dindo (CD) and survival. Patients received neo-adjuvant chemotherapy according to the respective Ewing sarcoma protocols. Depending on tumor location and organ infiltration, a multi-disciplinary surgical team was orchestrated to perform radical en-bloc resection and simultaneous chest wall repair. Results: Thirteen consecutive patients (seven boys and six girls) were included. Median age at presentation was 10.9 years (range 2.2-21 years). Neo-adjuvant chemotherapy (n = 13) and irradiation (n = 3) achieved significant reduction of the median tumor volume (305.6 vs. 44 ml, p < 0.05). En-bloc resection and simultaneous chest wall reconstruction was achieved without major complications despite multi-organ involvement. Postoperatively, one patient with infiltration of the costovertebral joint and laminectomy required surgical re-intervention (CD IIIb). 11/13 patients were treated with clear resections margins (R1 resection in one patient with infiltration of the costovertebral joint and marginal resection <1 mm in one child with multiple pulmonary metastases). All patients underwent postoperative chemotherapy; irradiation was performed in four children. Two deaths occurred 18 months and 7.5 years after diagnosis, respectively. Median follow-up for the remaining patients was 8.8 years (range: 0.9-30.7 years). The 5-year survival rate was 89% and the overall survival 85%. Conclusion: EWING specific oncological treatment and multi-disciplinary surgery performing radical en-bloc resections and simultaneous chest wall repair contribute to an improved survival of children with Ewing sarcoma of the chest wall.
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PURPOSE: Askin tumors are rare but highly malignant chest wall tumors, which require multimodal therapy including often extensive resection of the thoracic wall. This study evaluated the outcome of Askin tumor in seven patients with an interdisciplinary approach. METHODS: Patients' records, treated between 1994 and 2011, were reviewed retrospectively. Seven patients (three male, four female; mean age 12.3 years; range 2-21 years) were included. All patients received neoadjuvant chemotherapy. After reduction of initial tumor volume, radical tumor resection and thoracic wall reconstruction were performed. All survivors were evaluated in 2011 by clinical examination and lung function test. RESULTS: Five-year survival rate in our group of patients is 86 % and overall survival is 71 %. There were two mortalities. One patient passed away 7.5 years after the primary management, mainly attributed to tumor progression, which demanded aggressive surgical procedures and irradiation. Another patient died 18 months after the first diagnosis after several surgical interventions for recurrent multiple pulmonary metastases. Three years after the first diagnosis, one patient suffered from clear cell sarcoma of the contralateral kidney and developed a local recurrence of Askin 1 year later. The large chest wall defects arising after surgery have been successfully reconstructed using combination of latissimus dorsi muscle flaps and biomaterials. CONCLUSION: Data of pediatric patients with Askin tumor is scarce. Analysis of our seven patient series indicates that improved outcomes (71 % over all survival rate and 86 % 5-year survival rate) can be achieved by aggressive interdisciplinary management including radical surgery and chemotherapy. Chest wall stability can be achieved by utilization of local muscle flaps and biomaterials to cover surgical chest wall defects.
