Mandibular and para-mandibular tumors in children

Sixteen cases of mandibular tumors or paramandibular soft tissue tumors with mandibular involvement are reported. These include such rare mandibular tumors or tumor-like conditions as melanotic progonoma, intraosseous haematoma secondary to von Willebrand's disease, post-irradiation osteosarcoma, monostotic eosinophilic granuloma, aneurysmal bone cyst and osseous hemangiopericytoma. Three cases of cherubism, one of fibrous dysplasia or aggressive fibromatosis and one of central giant cell reparative granuloma are also reported. The soft tissue tumors comprise round cell sarcoma, parotid adeno-carcinoma with generalised metastases, embryonal rhabdo-myo-sarcoma, neuro-fibro-sarcoma and congenital cystic hygroma. In all the cases the disease was well advanced when the patient presented for X-ray examination. The specific X-ray diagnosis of mandibular and paramandibular tumors in childhood is more difficult than that of similar tumors in other parts of the body.Presented at the 24-th Annual Meeting of the American Society for Paediatric Radiology, San Francisco, California, USA, March 22–27, 1981; Presented at the 18-th Annual Meeting of the European Society of Paediatric Radiology, Oslo, Norway, May 20–22, 1981 and at the 32-nd Annual General Meeting of the Royal Australasian College of Radiologists, Christchurch, New Zealand, September 20–25, 1981     

Perinatal (Congenital and Neonatal) Neoplasms: A Report of 110 Cases

One hundred ten congenital and neonatal tumors encompassing a 25-year period are described and compared with similar published cases. Forty percent are classified as histologicallly malignant, and 65% of neonates with malignancies died. The types, frequency, and clinical features of neoplasms encountered in the perinatal period are markedly different from those observed in older children and adolescents. Their biological behavior and response to therapy are also dissimilar. Leukemia was responsible for the largest number of deaths followed by neuroblastoma and brain tumors.     

Rare tumors in childhood: Pathological aspects. Experience of the Kiel pediatric tumor registry

A wide variety of rare tumors can occur in childhood and adolescence. Tumors can be rare in any age group; common tumors of adults can appear in childhood and adolescence, and many rare tumors and tumor-like lesions show a predilection for younger age groups. Comparatively frequent tumors can present with rare histologic features or can occur in rare atypical locations, and, finally, seemingly common but, in fact, rare tumors have to be distinguished from truly common but rarely recognized tumors. Thus, the spectrum of rare tumors is broad, reflecting the fluent reality of life. © 1993 Wiley-Liss, Inc.     

Lipoblastoma in a four-year-old African child

A four-year old Sudanese child presented with a growing mass in the medial aspect of the right thigh. The mass appeared during the neonatal period. On clinical examination a diagnosis of lipoblastoma was entertained on the basis of the patient's age and the clinical features of the mass. The tumor was completely excised surgically. The clinical diagnosis of lipoblastoma was confirmed pathologically. Follow-up of the patient for 6 months postoperatively showed no evidence of recurrence.     

Bilateral germinoma of the basal ganglia

Germinoma arising in the bilateral basal ganglia is exceedingly rare, with only five cases reported to date. Owing to non-specific clinical findings and the frequent presence of ill-defined abnormalities without a definite tumor mass on neuroimaging, the diagnosis can be difficult. We describe a case in which magnetic resonance spectroscopy (MRS) findings suggested a tumor and supported the decision to perform biopsy of the lesion.     

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment

A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs. All‐cause mortality was highest in exocrine carcinomas (50%). Overall, 98.8% of patients with SPTs survived. PBL exhibited the highest recurrence rate (14.7%) within a mean of 23.5 months.     

Successful Treatment of a Recurrent Choroid Plexus Carcinoma with Surgery Followed by High-Dose Chemotherapy and Stem Cell Rescue

Choroid plexus carcinoma (CPC) is a rare central nervous system malignant tumor with a dismal prognosis, especially in the case of incomplete resection or recurrence. The authors report long-term survival of a 1-year-old patient with recurrent CPC and Li-Fraumeni syndrome with surgical resection and high-dose chemotherapy (HDC) consisting of single cycle of Busulfan and Thiotepa followed by autologous stem cell rescue without the use of radiation therapy. Remarkably the patient remains without evidence of recurrence 5 years after completion of therapy. Additional studies are necessary to determine the role of HDC and stem cell rescue in patients with recurrent CPC.     

BCOR‐CCNB3 fusion‐positive clear cell sarcoma of the kidney

Clear cell sarcoma of the kidney (CCSK) is the second most common malignant pediatric renal tumor. Two of the recurrent somatic alterations reported in CCSK are BCL‐6 corepressor (BCOR) internal tandem duplication (ITD) and YWHAE‐NUTM2B/E gene fusion. A minority of patients with CCSKs have other rare somatic alterations. We report two patients with CCSK showing BCOR‐CCNB3 (where CCNB3 is cyclin B3) fusion, who had similar clinical presentation of a large renal mass with tumor thrombus extending through the inferior vena cava into the right atrium and a favorable response to chemotherapy. We recommend BCOR‐CCNB3 fusion testing for all patients with CCSK who lack BCOR‐ITD or YWHAE‐NUTM2B/E gene fusions.     

Testicular tumors in childhood: a national report

The authors report preliminary data of an Italian multicentric, retrospective study of primary testicular tumors (PTT) in childhood involving 20 Italian departments of pediatric surgery. Forty cases of PTT were observed during a 10-year period (1977–1987), with yolk-sac tumors being the most frequent (45%). Most testicular tumors were first perceived as a unilateral testicular mass. Scrotal ultrasonography represents the main diagnostic investigation, together with serum marker levels.     

Non-midline endodermal sinus tumor in the head and neck region: A case report

Germ cell tumors, in particular teratomas, are some of the most commonly found tumors in childhood. Six percent of all germ cell tumors are located in the head and neck region. Endodermal sinus tumors (yolk sactumors) of the head and neck, exclusive of the central nervous system, are rare. This study reports a 20-month-old girl with a mass of 5 × 5 cm on the left temporal area and with bone destruction on CT. The histopathological examination of the excised mass revealed an endodermal sinus tumor. The serum AFP and the LDH levels were elevated at diagnosis. The patient was treated by the BEP protocol (bleomycin, etoposide, and cisplatin), six cycles every three weeks. Partial response was attained after the first and complete response after the fourth cycle. The patient, who is being followed up, has been in remission for five months. © 1993 Wiley-Liss, Inc.     

Critical review of the management of primary central nervous nongerminomatous germ cell tumors

Multimodal strategies have significantly improved the outcomes for patients with central nervous system nongerminomatous germ cell tumors. Two large cooperative group studies have recently reported much improved outcomes compared with historical series. However, a substantial proportion of patients still attain inadequate responses to initial chemotherapy prior to irradiation, with adverse impact upon survival; optimal induction chemotherapy regimens and radiotherapy strategies are as yet unidentified. Outcomes for patients with relapsed disease remain poor. There is an obvious need to incorporate molecular studies within prospective clinical trials that will likely lead to the incorporation of targeted, more effective future treatment strategies.     

Ependymal tumors in childhood

BACKGROUND: Ependymal tumors are classified as ependymoma (benign or low grade) versus anaplastic ependymoma (malignant or high grade). Ependymomas represent 5-10% of intracranial neoplasm in children. In this study, demographic data and the treatment results of pediatric patients with ependymal tumors, treated in a single institute, is reported. PATIENTS AND METHODS: Between 1989 and 2001, 40 (22 M/18 F) previously untreated patients with a median age of 5.5 years (3 months-15 years), of histologically proven ependymal tumors (except ependymoblastomas) were referred to the Institute of Oncology, University of Istanbul. The localization was supratentorial in 18, infratentorial in 20, both supra and infratentorial in two patients. Histologic subgroups were 18 ependymomas (43.6%), and 22 anaplastic ependymomas (56.4%). Total tumor resection was performed in 20 patients (50%), subtotal in 18 patients (45%), and biopsy only in 2 patients (5%). Postoperative treatment consisted of regional (8 patients) or craniospinal (CSI) (9 patients) radiotherapy (RT) in patients with ependymoma; regional (7 patients) or CSI RT (14 patients) with chemotherapy (ChT) in patients with anaplastic ependymoma; ChT only (1 patient) in patients less than 3 years of age. The standard technique for posterior fossa irradiation was parallel-opposed lateral fields and total dose was 45-54 Gy. Between September 1989 and May 1991 patients received regimen A, which consisted of RT followed by eight-in-one ChT, given every 4 weeks for eight courses. Patients who were treated between June 1991 and July 1994, received regimen B, which included two courses of postoperative "VEC" (vincristine, etoposide, cisplatin) ChT, administered every 3 weeks, followed by RT applied with low dose concomitant cisplatin used as a radiosensitizer. Patients with objective response to postoperative "VEC" continued to have "VEC" after completion of RT for six more courses. From August 1994 on, patients received regimen C, consisting of RT and concomitant infusion of cisplatin followed by "VCPCU" (vincristine, cyclophosphamide, procarbazine, lomustine) administered every 4 weeks for eight courses. RESULTS: A total of 40 patients were included in the outcome and survival data. The 5-year overall survival (OS) rate was 64.9%, and the 5-year progression-free survival rate was 50.8% for the whole series. Median time for progression or relapse was 24.3 months and there were 19 patients (43.6%) with relapse or progression. Non-metastatic patients (P = 0.0008, 5-year OS rate was 82% vs. 29%), and totally resected patients (P = 0.01, 5-year OS rate was 80% vs. 55%), and > or =3 years of age (P = 0.04, 5-year OS rate was 75% vs. 38%) had significantly better outcome. CONCLUSIONS: The majority of complete responders were patients who had total tumor removal. Treatment failure occurred mainly within the first 2 years, and outcome was dismal for patients who relapsed or had progressive disease. The median age at diagnosis is 6 years in our patient group; younger children (less than 3 years old) have less favorable outcome. There was no significant difference in survival or progression-free survival between the two histologic subtypes.     

OLIGODENDROGLIOMA ARISING IN A MATURE CYSTIC OVARIAN TERATOMA IN A CHILD

In childhood mature cystic teratomas are the most common type of ovarian germ cell tumors. Tumors of neuroepithelial origin are extremely rare in mature teratomas. To the authors’ knowledge, this is the first case report oligodendroglioma arising in a mature cystic ovarian teratoma in children. A 13-year-old girl presented with a history of pelvic pain for 2 months. An ultrasound examination showed a monolateral multicystic, calcific ovarian mass, measuring approximately 6 × 5 cm, arising in the left over and adnexia, suggesting a teratoma. An exploratorative laparotomy and ooferectomy were performed. On histopatological examination, various samples from cystic areas had mature tissues from all 3 germ cell layers, including skin, bone, bronchial structures, and cerebellum. Sections from solid areas showed uniform population of monotonous, rounded cells with indistinct cytoplasm and having perinuclear halo (fried-egg appearance) on a fibrillary, neuropilic background. Microcalcifications as calcospherites were noted in the stroma. Based on the typical morphological features, the cased was diagnosed as mature cystic teratoma with low-grade oligodendroglioma. To the authors’ knowledge, there are a few reports in the literature of an oligodendroglioma arising in an ovarian teratoma in adults and this presented patient is the first case in childhood.