Salvage Treatment of Local Recurrence in Esthesioneuroblastoma: A Meta-analysis

Esthesioneuroblastoma has a local recurrence rate of ～30%, but no standard regimen for salvage of local recurrence has been established. We report data from 678 patients from series published since 1990, with a risk reduction analysis of salvage with surgery, radiation, or combined surgery and radiation therapy. We found a 28.5% rate of local recurrence after treatment, and a 42.6% rate of successful salvage with surgery, radiation, or combined treatment. The odds ratio for successful salvage, defined as disease-free survival for at least 1 year, was not significantly different for combined surgery and radiation versus surgery alone or radiation alone or for surgery alone versus radiation alone. The salvage odds ratio for combined surgery and radiation therapy versus radiation therapy alone, 3.5, approached, but did not reach statistical significance. This study reveals a reasonable rate of successful salvage of local esthesioneuroblastoma recurrence using surgery, radiation, or combined surgery and radiation.     

Bladder papilloma in a child. Case report.

A case of bladder papilloma in a 12-year-old boy is presented. He had had painless, intermittent hematuria. An excretory urogram revealed a filling defect in the bladder. A walnut-sized, pedunculated papilloma was removed by transvesical extirpation. Histological examination showed a papillary bladder tumour, grade 1 (Bergkvist et al., 1965). Our patient also had a Cushing syndrome and later a bilateral adrenalectomy was done. The coexistence of two such rare conditions may not be purely coincidental. Control cystoscopies have shown no sign of tumoral recurrence.     

Prognostic Utility of Hyams Histological Grading and Kadish-Morita Staging Systems for Esthesioneuroblastoma Outcomes

Esthesioneuroblastoma (ENB) is derived from the specialized olfactory neuroepithelium. Hyams grading and Kadish staging have been used to prognosticate and to guide treatment decisions. In this study, we sought to validate the prognostic utility of these systems in a large ENB cohort. We retrospectively analyzed the records of patients with ENB who had been evaluated and treated at our institution. The association of grade and stage with prognostic outcome was assessed; the Kaplan–Meier estimator was used to generate 5-year OS and DFS curves. Out of 124 cases we identified, 121 were assessed for grading and 109 for staging. Review of the tissue samples revealed that 62 % of tumors were low grade (I/II) and 21 % were high grade (III/IV); 17 % of tumors were metastasis. The OS rate was 75 % at 5 years. The DFS was 60 % at 5 years. The OS was significantly worse for metastatic ENB (low-grade ENB vs metastatic ENB p = 0.01598); the DFS was significantly worse for high grade versus low grade ENB. Of the 109 cases that had been staged, 16 % were stage A, 33 % stage B, 43 % stage C, and 8 % stage D. In the A, B, and C groups, there were no significant differences between recurrence, distant metastasis, or 5-year survival rates. Statistical significance was not reached with the T, N, M and overall staging system. Age cutoff of 65 years reliably predicted OS. High grade of ENB was significantly associated with poor outcome, while advanced stage was not associated with poor outcome in this large cohort. Grading should certainly be considered in prognostication and treatment decisions for ENB.     

Sciatic nerve entrapment in a child. Case report

A child was brought for evaluation of signs of unilateral chronic progressive sciatic nerve dysfunction found to be due to nerve entrapment in the thigh by a fibrovascular band. Sectioning of the band was followed by marked improvement in the nerve function. Compression by a band is a rare but treatable cause of sciatic neuropathy.     

Noncontiguous bilateral esthesioneuroblastoma: a case report.

Esthesioneuroblastoma is a rare malignant neoplasm arising from the olfactory epithelium. This tumor has a tendency to originate from one side of the nasal cavity or paranasal sinuses with frequent extension into the contralateral nasal cavity through direct invasion. A review of the literature reveals numerous case reports describing esthesioneuroblastoma with unilateral or bilateral involvement; however, there have been no known reports of bilateral noncontiguous (multifocal) esthesioneuroblastoma. We present a unique case of a patient diagnosed with two separate primary esthesioneuroblastomas who was managed with preoperative radiation followed by surgical resection of the tumor. This case illustrates that esthesioneuroblastoma must remain as part of the differential diagnosis for a multifocal, noncontiguous intranasal and paranasal sinus mass.     

Brainstem epidermoid cyst in a child. Case report

A 10-year-old girl presented with a brainstem epidermoid cyst manifesting as dysphagia and post-prandial vomiting. Magnetic resonance imaging disclosed a cystic mass in the brainstem with associated hydrocephalus. She was treated under a presumptive diagnosis of abscess until the definitive histological diagnosis was established at the fourth admission. In spite of four interventions, she remained in good condition with minimal cranial nerve pareses. Subtotal removal of the epidermoid cyst in the brainstem results in recurrence within a relatively short period, especially in children. Total resection of the tumor is the optimum treatment. The differential diagnosis includes abscess, cystic glioma, and neurenteric cyst.     

Giant cystic cavernoma in a child. Case report

A totally cystic giant cavernous hemangioma is described in a 3-year-old girl. The clinical presentation and computerized tomography findings were both unique. The patient was successfully treated by surgery.     

Recurrent Lhermitte-Duclos disease in a child. Case report

A case of recurrent Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) in a child is described with a summary of the clinical presentation and associated malformations, and a review of other cases reported in the literature. The histological examination and electron microscopic findings, with special reference to the cytological changes found during evaluation of the recurrence, are presented. Theories regarding the pathogenesis of Lhermitte-Duclos disease are reviewed.     

Diffuse leptomeningeal involvement by a ganglioglioma in a child. Case report.

Gangliogliomas are tumors composed of neuronal and glial elements that typically grow slowly by expansion only. This report describes a 20-month-old girl with a ganglioglioma that extensively involved the subarachnoid space; microscopic foci of tumor were found in the brain and spinal cord. Despite chemotherapy and radiation therapy, the child died 5 months after diagnosis. Molecular genetic analysis showed loss of chromosome 17p DNA sequences in the tumor tissue.     

Stereotactic pallidotomy in a child with Hallervorden-Spatz disease. Case report

The authors present a case of Hallervorden-Spatz disease (HSD) in a 10-year-old boy treated with stereotactic pallidotomy for control of severe dystonia. Hallervorden-Spatz disease is a rare type of neuraxonal dystrophy that can be familial or sporadic. This is the first case of HSD reported in the literature in which a pallidotomy was performed. The patient had progressively worsening dystonias and spasms that prevented useful function of his entire right side and eventually threatened his respiratory ability. Pre- and postoperative magnetic resonance images are presented along with electrophysiological recordings made in the globus pallidus at the time of surgery. Functional improvement in the use of the patient's limbs and relief from the painful dystonia were observed. Stereotactic pallidotomy should be considered as a potential treatment in the management of HSD.     

Neurilemmona of the median nerve in a child. Case report

A case of neurilemmoma of the median nerve in a child is reported. The rarity, the contribution to the diagnosis of the newer imaging methods, especially MRI, as well as the good prognosis after a careful enucleation of the tumor, are emphasized.     

Congenital abdominal aortic aneurysm in a young adult. Case report.

A 30-year-old man presented with threatened rupture of an infrarenal aortic aneurysm. Clinical and laboratory investigations revealed no traumatic, atherosclerotic, infectious or inflammatory cause. Histologic examination of the aortic wall showed cystic degeneration of the media. Consequently the case could be classified as congenital abdominal aortic aneurysm in an adult.     

Esthesioneuroblastoma: The Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital Experience with Craniofacial Resection, Proton Beam Radiation, and Chemotherapy

Objectives: To determine the efficacy of craniofacial resection and proton radiation for the management of esthesioneuroblastoma (ENB). Design: A retrospective chart review was performed of all patients presenting with ENB and completely managed at the Massachusetts General Hospital (MGH) and the Massachusetts Eye and Ear Infirmary (MEEI) from 1997 to 2006. Setting: A tertiary referral center. Main Outcome Measures: Disease-free and overall survival. Participants: All patients presenting with ENB and completely managed at the MGH and the MEEI from 1997 to 2006. Results: Ten patients were identified with a median follow-up time of 52.8 months. Average age at presentation was 45 years. Nasal obstruction was the most common presenting symptom. Three patients presented with Kadish stage B disease and seven with stage C. No patient had evidence of cervical or metastatic disease at presentation. Seven patients were treated with craniofacial resections (CFR) followed by proton beam radiation with or without chemotherapy. Three patients were treated with initial chemotherapy with no response. They subsequently underwent CFR followed by proton beam radiation. The 5-year disease-free and overall survival rates were 90% and 85.7%, respectively, by Kaplan-Meier analysis. No patient suffered any severe radiation toxicity. Conclusion: ENB can be safely and effectively treated with CFR followed by proton beam irradiation. Proton irradiation may be associated with less toxicity than photon irradiation. The role of chemotherapy remains unclear.     

Tracheal obstruction due to congenital tracheomalacia in a child. Case report.

Congenital tracheal obstruction, though not notably uncommon in infancy, is rarely due to isolated tracheomalacia, especially when characterized by complete absence of cartilaginous rings. A 5-year-old boy underwent successful tracheal resection and anastomosis following severe tracheal obstruction due to aplasia of cartilaginous rings.     

Dumbbell lymphoma of the cervical spine in a child. Case report.

Primary or secondary spinal involvement of lymphoma is a rarely reported entity. An eleven-year-old girl with primary cervical dumbbell non-Hodgkin's lymphoma (NHL) was presented. We could not found any such growth pattern of primary or secondary NHL'S in the literature. For this reason, we reviewed shortly pertinent literature and discussed the pathophysiologic, diagnostic and prognostic features of the lesion with treatment modalities.