Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country.

Incesoy-Özdemir S, Ertem U, Sahin G, Bozkurt C, Yüksek N, Ören AC, Balkaya E, Alkan A. Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country. Turk J Pediatr 2017; 59: 410-417. Germ cell tumor (GCT) is a rare malignancy accounting for 2-3% of all pediatric tumors. The overall survival rate of children and adolescents with GCT is more than 80% after adopting combined therapy. The aim of this study is to review clinical presentation, management, and outcome in a single-center series with extracranial GCT. Clinical characteristics, pathologic presentations, and survival outcomes of 101 children with GCT, treated at our hospital from 1988 to 2011, were analyzed. Sixty-two of patients were female and 39 of them were male. Fifty-eight (57%) patients had gonadal tumor (24 testicular, 34 ovarian), 43 (43%) extragonadal. Histologically, teratomas were found most frequently (26 mature, 10 immature), followed by yolk sac tumors (n: 33), mixed malignant tumors (n: 13), embryonal carcinoma (n: 10), disgerminoma (n: 8) and seminoma (n: 1). Twenty-six patients were diagnosed as mature teratoma and we excluded them in the evaluation of staging and survival. Five-year overall and relaps-free survival were 80.3% (mean follow-up time: 215.8 months) and 73.4% (mean follow-up time: 176.2 months), respectively. Five-year survival rates were 93.2% and 90.2% in malign GCTs diagnosed after 1999.

Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases.

Balkaya E, Bozkurt C, Aksu AE, Özmen S, Incesoy-Özdemir S, Sahin G. Ewing`s sarcoma of the mandible misdiagnosed as periodontal inflammation: Report of three cases. Turk J Pediatr 2017; 59: 704-707. Ewing`s sarcoma (ES) is the second most common childhood primary malignant tumor of the bone. The most popular locations of ES are long bones and pelvis. The involvement of the mandible is very rare in childhood. In last 10 years, we met with three cases of ES of the mandible in our department. Initially the patients had symptoms similar to periodontal inflammation. The involvement of the mandible might be considered with periodontal inflammation in its initial stages, what frequently leads to delayed treatment. Although this tumor has an aggressive clinical behavior and rapid growth, early diagnosis can reduce patient`s morbidity and mortality and thus it is important to distinguish from periodontal inflammation.

A subcutaneous paraspinal yolk sac tumor in a child.

A 3-year-old girl underwent a surgery at an external center on July 2011 for a swelling in the left lumbar paravertebral subcutaneous region. The mass was completely excised and the pathologic diagnosis was a yolk sac tumor (YST). Laboratory tests revealed a serum α-fetoprotein level of 278 IU/mL. Investigations using bone scintigraphy and magnetic resonance imaging revealed a scar tissue at the surgical site and lesions indicating metastasis at the lumbar first, second, third, and fifth vertebra. The patient was administered 5 cycles of PEB (cisplatin, etoposide, bleomycin) treatment. The serum α-fetoprotein was 3 IU/mL after the treatment. The lumbar magnetic resonance imaging and bone scintigraphy results were normal. The patient continues to be in remission since June 2012. YSTs are most commonly seen in the testis, ovary, and sacrococcygeal regions. Atypical locations have been reported with the primary lesion in the stomach, diaphragm, omentum, sino-nasal region, cranial base, lungs, vagina, and penis. Our case is probably a YST with an atypical location derived from preliminary cells left under the skin because of a migration defect.

A rare type of secondary cancer in a child with acute lymphoblastic leukemia: malignant fibrous histiocytoma.

Secondary cancers which are related with treatment of childhood acute lymphoblastic leukemia (ALL) is a significant problem with longer term. For development of secondary cancer after treatment, the latency period varies between 5 and 10 years. In this case, a 13 year-old-boy diagnosed as high-risk ALL was treated with chemotherapy and prophylactic cranial radiotherapy at a dose of 1800 cGy. Six years after the end of treatment he developed a 5 × 5 × 4 cm mass at the right temporal region of the cranium. The mass was excised totally with clear surgical margin. Pathology of mass has been diagnosed as malignant fibrous histiocytoma (MFH), recently referred to as an undifferentiated pleomorphic sarcoma (UPS). After treatment of childhood ALL, reported cases of secondary MFH is extremely rare in the literature. Herein we present a case of MFH/UPS that developed as a secondary cancer 6 years after the end of ALL treatment.

The relationship between cerebrospinal fluid osteopontin level and central nervous system involvement in childhood acute leukemia.

The aim of this study was to evaluate the relationship between cerebrospinal fluid (CSF) osteopontin (OPN) levels and central nervous system (CNS) involvement in children with a diagnosis of acute leukemia. The study sample consisted of 62 patients who had been diagnosed with acute leukemia. The control group consisted of 16 patients that had presented and had no malignant disease, CNS infection or chronic disease. CSF OPN levels were studied with enzyme-linked immunosorbent assay (ELISA) method. The mean CSF OPN level was 32.76±49.22 ng/ml in the patients at the time of diagnosis and 14.93±6.84 ng/ml in the control group (p>0.05). The mean CSF OPN level was 27.68±32.73 ng/ml at the time of diagnosis in the group without CNS involvement and 53.48±89.21 ng/ml in the group with CNS involvement (p>0.05). However, the CSF OPN level at the time of CNS relapse in patients who developed CNS involvement during follow-up (127.4±52 ng/ml) was significantly higher than in the group without CNS involvement at diagnosis and follow-up (mean CSF OPN level: 27.68±32.73 ng/ml) (p<0.001). The analysis of CSF OPN levels at the time of diagnosis-before relapse and at the periods of relapse and remission in patients who had CNS involvement at diagnosis and/or follow-up revealed statistically significant differences between the time points (p<0.001). High CSF OPN levels in childhood acute leukemia patients may be used as evidence for CNS involvement, and any increases found in CSF OPN levels may be a preliminary predictor for CNS involvement.

Oropharyngeal tularemia mimicking tumoral relapse in a patient with Hodgkin lymphoma in remission.

Tularemia is a zoonotic disease caused by Francisella tularensis. The clinical forms mostly depend on the port of entry into humans. Ingestion typically results in the oropharyngeal form and is associated with symptoms such as fever, pharyngitis, cervical lymphadenitis, and suppuration. In this report, we describe a child treated for Hodgkin's disease presenting six years later with a left cervical lymphadenopathy mimicking a relapse.