Mark L. Kayton

Experience with 31 sentinel lymph node biopsies for sarcomas and carcinomas in pediatric patients

Few data exist regarding techniques, indications, and outcomes for sentinel lymph node biopsy in pediatric patients with sarcomas and carcinomas.

Primary lung adenocarcinomas in children and adolescents treated for pediatric malignancies.

Introduction: Primary lung adenocarcinoma is extremely rare in the pediatric age group. There have been anecdotal reports of lesions that are histologically indistinguishable from adult-type pulmonary adenocarcinoma in young patients after treatment for nonpulmonary cancers. Herein, we present clinical, histopathologic, and molecular data on eight such cases. Methods: Histopathologic evaluation of the tumors was performed according to the World Health Organization classification. Molecular studies for EGFR and KRAS mutations were performed on six patients with sufficient material. Results: All eight patients were never smokers, four males and four females. Median age at nonpulmonary cancer diagnosis was 14 years (range, 3–23 years). Pulmonary adenocarcinomas were diagnosed at a median age of 15 years (range, 10–24 years); tumors were 0.1 to 2.0 cm in size and in some cases coexisted with metastases from the original cancer. Retrospective review showed that in at least three patients, the nodules were radiographically present before chemotherapy. Of six patients whose tumors were tested for common EGFR and KRAS mutations, two were positive for the former and one for the latter. At a median follow-up of 11 months (range, 2–29 months), six patients remained well without lung nodules and two had additional small, peripheral lung nodules that have not been biopsied. Conclusions: Pulmonary lesions found in young patients with pediatric cancers can be histologically indistinguishable from lung adenocarcinoma seen in adults, may display typical adenocarcinoma-associated mutations of EGFR and KRAS, and may precede the administration of cytotoxic chemotherapy.

Pediatric radical abdominal trachelectomy for anaplastic embryonal rhabdomyosarcoma of the uterine cervix: an alternative to radical hysterectomy.

Rhabdomyosarcoma arising in the female genital tract carries 5-year survival in excess of 80%, but lifelong infertility may be a consequence of local control strategies. We present the technique and outcome for a fertility-sparing, radical abdominal trachelectomy in a 12-year-old girl with anaplastic, embryonal rhabdomyosarcoma involving the uterine cervix. The patient had presented to our center after the piecemeal resection of a uterine cervical mass; because of concern about microscopic residual disease, we classified her as group II-A according to the Intergroup Rhabdomyosarcoma Study system. Staging studies excluded the presence of distant disease. The patient received 4 cycles of multiagent chemotherapy and then underwent radical abdominal trachelectomy, with removal of the uterine cervix, parametria, vaginal cuff, and regional lymph nodes. Microscopically, the specimen showed treatment effect and no residual tumor. Regional nodes were negative. Radical abdominal trachelectomy, which has not been previously reported for rhabdomyosarcoma, has appeared to secure local disease control in this case while preserving the patients future fertility potential. In properly selected cases of rhabdomyosarcoma of the uterine cervix, where involvement of the uterus proper is not present, radical abdominal trachelectomy may be an attractive fertility-sparing alternative to radical hysterectomy.

Pediatric radical abdominal trachelectomy for solitary fibrous tumor of the uterine cervix

OBJECTIVE Solitary fibrous tumor is a rare mesenchymal tumor initially described arising in the pleura but now reported in many other thoracic and extrathoracic sites. Here we report the first case of a 14-year-old girl who was discovered to have a large solitary fibrous tumor of the uterine cervix, an unusual site for these tumors, to highlight the diagnosis and management of these tumors. METHODS Case report and review of the literature on extrapleural solitary fibrous tumors. RESULTS The median age of occurrence is in the late 50s and there is only one previous report in the literature of a solitary fibrous tumor in a patient less than 18 years old. Most patients will be cured by complete surgical resection though late local and distant relapse will occur in a minority of patients, many of whom have indicative histological features. Complete surgical resection of this tumor arising in the uterine cervix, was achieved by performing a radical abdominal trachelectomy. CONCLUSIONS Review of the literature confirms the good long-term outcome of the majority of these patients with complete surgical resection but emphasizes the importance of close long-term follow-up.

Experience with aortic grafting during excision of large abdominal neuroblastomas in children

BACKGROUND Total or near total resection of high-risk, stage 4 abdominal neuroblastoma has been correlated with improved local control and overall survival but may be complicated by vascular injury. We describe our experience in the management of significant aortic injuries during this procedure. METHODS With the institutional review board waiver, medical records of children who had major abdominal aortic reconstruction during neuroblastoma resection from 1996 to 2006 were retrospectively reviewed. RESULTS There were 5 children with aortic grafting: 3 girls and 2 boys. Mean age at surgery was 7.2 years (range, 16 months to 17 years). Two children were operated on for recurrent retroperitoneal disease. Tumor encasement of the aorta was seen in all children. In 3 children, the injury occurred during dissection of paraaortic and interaortocaval lymph nodes below the level of the renal arteries. In the remaining 2 children, injury occurred early during mobilization of the tumor. Three polytetrafluoroethylene tube grafts and 1 on-lay patch graft were used to repair the 4 distal aortic injuries. One 4-year-old female with aortic and renal arterial injuries was managed with an aortic Dacron tube graft and a polytetrafluoroethylene tube graft for the renal artery. The mean period of follow-up is 28 months after aortic graft (range, 3 months to 10 years). Total colonic ischaemia, transient acute tubular necrosis, and duodenal perforation were seen in one child, who needed subtotal colectomy and ileostomy. Another child with an omental patch over the graft had a transient duodenal obstruction, which was managed conservatively. There were no other complications, and 4 of the 5 children are disease-free to date. One child at 10 years after his distal aortic tube graft remained asymptomatic with normal distal blood flow on magnetic resonance angiogram and with normal growth. CONCLUSION The neuroblastoma surgeon should be prepared to perform aortic and vascular reconstruction. Aortic encasement, preoperative radiation therapy, and reoperative surgery were observed in these patients and may be risk factors.

Splenic infarction and subsequent splenic rupture in a patient with paroxysmal nocturnal hemoglobinuria and heparin‐induced thrombocytopenia

We describe a patient with paroxysmal nocturnal hemoglobinuria (PNH) and no previous history of thrombosis who presented with hepatic venous thromboses and subsequently developed splenic infarction and rupture requiring splenectomy while on anticoagulation therapy for the hepatic thromboses. The patients anticoagulation was complicated by heparin‐induced thrombocytopenia (HIT) highlighting the unique management challenge presented by PNH in combination with HIT. Pediatr Blood Cancer 2009;53:472–474.

Eradication of cryptosporidium from a defunctionalized colon limb by refeeding stoma effluent

Over the last 40 years, cryptosporidium has increasingly been recognized as a cause of acute self-limiting diarrhea in normal hosts. In the immunocompromised patient, cryptosporidium may cause severe illness with prolonged diarrhea and malabsorption. Pharmacologic therapy of cryptosporidium relies on adequate delivery of drug metabolites to the colon. Here we describe a patient who developed toxic megacolon during induction therapy for leukemia, requiring ileostomy formation to proceed with leukemia treatment. Although the megacolon resolved promptly, the resulting isolation of the colon from the fecal stream prevented luminal delivery of active metabolites of anti-protozoal drugs, resulting in persistent cryptosporidiosis. Refeeding of the ileostomy output into the colon effectively eradicated cryptosporidium from the colon and permitted closure of the ileostomy.

Vascular Endothelial Growth Factor Expression and Pulmonary Development in Murine Fetal Heart-Lung Culture

Vascular Endothelial Growth Factor Expression and Pulmonary Development in Murine Fetal Heart-Lung Culture