Sabri Acar

Electrocardiographic findings after 5-HT3 receptor antagonists and chemotherapy in children with cancer

The antiemetic efficacy of serotonin‐type 3 (5‐HT3) receptor antagonists has been found to be superior to older antiemetic drugs in cancer patients. Following the administration of these agents, changes in ECG parameters and increased or decreased heart rates have been demonstrated, but there is no sufficient data in children with cancer who are treated with cytotoxic agents. The objective of this study is to evaluate the ECG changes after administration of 5‐HT3 receptor antagonists and chemotherapeutic agents in children with cancer.

Protective effect of vitamin A on ARA-C induced intestinal damage in mice.

Background Cytarabine (ARA-C) has been used for many years in the treatment of patients with leukemia and lymphoma. Gastrointestinal ulceration and mucositis are two of the well-known side effects of ARA-C. We set out to investigate whether vitamin A (VA) can help prevent ARA-C-induced mucosal lesions in mice. Materials and methods Mice were divided into 5 groups. Group I (control group) received only saline; group II received ARA-C plus saline; group III received ARA-C plus VA; group IV received ARA-C plus a lipid solution, and group V received VA alone. VA (5000 IU/kg) was administered orally to the mice once daily for 7 days. ARA-C (3.6 mg) was administered intraperitoneally for 5 days to groups II, III and IV, starting on the third day of VA treatment. Intestinal segments from the proximal end of the jejunum of treated mice were isolated. Results There was improved mucosal integrity, less necrosis and increased villus length with advanced mucosal proliferation in crypts in the VA plus ARA-C group when compared to the ARA-C groups without VA. Conclusion We conclude that VA has a protective effect against ARA-C-induced mucosal damage in mice.

Hair selenium status in children with leukemia and lymphoma.

Selenium (Se) is a trace element contributing to the structure of antioxidant system that saves cells from reactive oxygen species. Low serum Se levels have been reported in pediatric and adult patients with cancers. On the other hand, hair Se levels, predicting the long-term body Se status, have been reported in only adult patients with cancer. The aim of the study was to investigate the hair Se status in children with newly diagnosed lymphoid malignancies and the relation between malnutrition and Se deficiency. Thirty patients with leukemia (n=17) and lymphoma (n=13), and 25 healthy controls were enrolled to the study. Se was determined with atomic absorption spectrophotometrical method. Hair Se levels of the patients were significantly lower than those of control group [666.96±341.46 ng/g vs. 1019.22±371.83 ng/g (P<0.001)]. Children with lymphoma had lower Se than the children with acute lymphoblastic leukemia but not statistically significant [547.03±283.67 ng/g vs. 758.67±361.05 ng/g (P>0.05)]. Malnourished patients (11/30) had lower hair Se levels (483.51±235.55 ng/g) than those of the controls (P=0.036), whereas the Se levels of the patients who had no malnutrition (773.17±352.92 ng/g) were also lower than those of the controls but not statistically significant (P=0.053). There was no correlation between age, sex, and the hair Se levels. In this study, we found that hair Se levels of the children with leukemia and lymphoma, especially those of malnourished patients, were lower than those of controls. Additional studies are needed to determinate whether low levels of hair Se may play a role in carcinogenesis.

Bilateral adrenal cystic neuroblastoma with superior vena cava syndrome and massive intracystic haemorrhage

Bilateral cystic adrenal tumours are a rare presentation of neuroblastoma. Intratumoural haemorrhage is a frequent finding in neuroblastoma, but is rarely symptomatic. We present an 11-month-old girl with predominantly cystic bilateral neuroblastomas and distant lymph-node metastasis. Massive intracystic haemorrhage and superior vena cava (SVC) syndrome were ominous prognostic factors, leading to death. Large tumours with intracystic haemorrhage might require a conservative approach.

MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY OF THE FEMUR

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that often occurs during the first year of life. More than 90% of MNTIs occur in the head and neck region, with most on the anterior part of the maxillary ridge. MNTI has also been reported in the mediastinum, thigh, foot, shoulder, and gonads. Here the authors report a rare case of MNTI of the femur in a 3-month-old child.

ACQUIRED VON WILLEBRAND SYNDROME IN A PATIENT WITH EWING SARCOMA

Acquired von Willebrand syndrome is a rare bleeding disorder with clinical and laboratory features closely resembling to hereditary von Willebrand disease. The syndrome may accompany various conditions, including malignant disorders, most often with Wilms tumor and adrenal cell carcinoma. In this report, the authors present a patient with AvWS in association with Ewing sarcoma for the first time in the literature. The abnormal bleeding tendency was successfully treated with fresh frozen plasma and did not recur after the first two courses of chemotherapy with clinical improvement.

SACROCOCYGEAL TERATOMA WITH SARCOMATOUS DIFFERENTIATION IN A CHILD

Most of the extragonadal teratomas are located in the sacrococygeal region. Teratoma with malignant sarcomatous differentiation is a rare form of germ cell tumor. The authors describe a 5-year-old-girl with sacrococygeal teratoma in which sarcomatous elements were observed. The patient was treated with complete surgical excision and adjuvant chemotherapy according to sarcoma protocols.

Second primary rhabdomyosarcoma of bone after osteosarcoma.

To the Editor: Secondary malignant neoplasms are infrequent but extremely serious events following therapy for primary cancers, especially Hodgkin lymphoma [1]. Secondary cancers after osteosarcoma have also been infrequently reported and most of them are central nervous system tumors and leukemias; sarcomas and carcinomas have been rarely reported [2–7]. Here we report a case of secondary rhabdomyosarcoma of the humerus after 5 years of lower extremity osteosarcoma. A 12-year-old girl was admitted with the complaint of swelling and pain just over the right knee. Plain X-ray showed lytic and radiolucent areas on the 1/3 distal end of the right femur. Magnetic resonance imaging (MRI) was concordant with an osteosarcoma arising from the distal end of the right femur and histopathological examination of biopsy material verified the diagnosis. Computerized tomography (CT) of the chest and bone scan revealed no metastases. After preoperative chemotherapy of T10 protocol [7] including four doses of high-dose methotrexate (12 g/m), limb sparing surgery with the implantation of a modular prosthesis was done. Histopathological examination of the resection material revealed necrosis of less than 10% of the tumor. Adjuvant chemotherapy regimen including cisplatin (instead of methotrexate), bleomycin, cyclophosphamide, dactinomycin, and doxorubicin was given uneventfully (T10A) for 4 months. After 5 years from the initial diagnosis of osteosarcoma, she had a complaint of head and right humerus pain without any soft tissue swelling. Cortical degeneration of the tuberculum majus and medullary lytic areas were seen on plain X-ray (Fig. 1A). CT revealed irregular lytic degeneration on the lateral burden of the head of humerus with normal surrounding soft tissues, but the fascial plane was irregular on the posterior part of the humerus (Fig. 1B). She was admitted again after a month with a swollen shoulder. Hypodense defects beginning from epiphyseal plaque extending to the metaphysis and around the head of humerus were seen on T1 series on MRI. There were irregularities beginning from the head of humerus to the metaphysis and soft tissue component extending to the paraosteal area on T2 series of MRI. Bone scan showed diffuse osteoblastic activity in the proximal region of the right humerus and there was hypoactivity on the head of humerus due to lytic areas. CTand bone marrow examination were normal. Trucut biopsy revealed high-grade malignant mesenchymal tumor. An operation was performed that consisted of the resection of the upper 1⁄2 part of the humerus and implantation of a prosthesis. Histopathological examination was consistent with a pleomorphic rhabdomyosarcoma (Fig. 2A); immunohistochemical studies revealed positive staining for vimentin and desmin and focal positive staining with CD68, while S100 and CD34 staining were negative. Rhabdomyoblasts with positive staining with desmin are shown in Figure 2B. She is on chemoradiotherapy with no evidence of recurrence at this time. Secondary malignant tumors developing after osteosarcoma have been reported as series or single case reports. Most of them are CNS tumors and leukemias but various malignant tumors including malignant fibrous histiocytoma, melanoma, chondrosarcoma, breast cancer, stomach cancer, carcinoma of colon and rectum, liposarcoma, breast carcinoma, mucoepidermoid carcinoma, myelodysplatic syndrome, non-Hodgkin lymphoma, high-grade pleomorphic sarcoma and leiomyosarcoma have also been reported [2–7]. The secondary cancer of our patient is interesting because of the bone origin in association with histopathological and immunohistochemical examination concordant with pleomorphic rhabdomyosarcoma. Some cases of multicentric osteosarcomas may recur in a different location after a long time period (metachroneous osteosarcoma) and may be confused with secondary cancer [8]. Genetic predisposition may increase the incidence of primary and secondary cancers in patients and close relatives. In our patient, there was no evidence of a familial cancer predisposition, including no history of retinoblastoma, osteosarcoma or Li–Fraumeni syndrome in family

Çocukluk Çağı Ewing Sarkomlu Olguların Retrospektif Değerlendirilmesi

Bu calismada Cocuk Hematoloji-Onkoloji Bilim Dali’nda takip ve tedavi edilen 27 Ewing Sarkomlu olgunun epidemiyolojik ve klinik ozellikleri, tedavi sonuclari, uzun donem komplikasyonlari retrospektif olarak incelenmistir. Olgularin (n=27) ortalama tani yasi 11.36 ±3.98 yil ( 8 ay- 17.9) olup, erkek/kiz orani 1.7 olarak saptandi. En sik basvuru yakinmalarinin lokalize agri (%96.3) ve sislik (%100) oldugu goruldu. Primer tumor bolgesi 16 olguda (%59.3) uzun kemiklerde, 11 olguda (%40.7) aksiyel kemiklerde saptandi. Primer lezyonun yerlestigi kemikler sirasiyla femur (%26), tibia (%14.8), pelvis (%14.8) ve fibula (%11.1) idi. Basvuru aninda 3 olguda ( %11.1 ) kemik iligi tutulumu tespit edildi. Toplam 9 olguda (%33.3) tani sirasinda uzak yayilim saptandi. 23 olguya (%92) neoadjuvan kemoterapi, 17 olguya (%77.2) adjuvan kemoterapi uygulandigi tespit edildi. Olgularin 13’une ekstremite koruyucu cerrahi, 2’sine amputasyon yapildigi saptandi. Radyoterapi olgularin %65.3’unde uygulandi (n=17) ve radyoterapi yeri 11 olguda primer tumor bolgesine, 5 olguda metastaz bolgesine, 1 olguda hem primer tumor hem metastaz bolgesine yonelik idi. Olgularinin ortalama ve ortanca izlem sureleri sirasiyla 35.5 ve 19 ay (en fazla 355 ay) idi. Sag kalim analizleri sonucunda toplam sag kalim orani %22.4±10.9 olarak saptandi. Retrospective assessment of patients with pediatric Ewing’s sarcoma In this study, 27 patients with Ewing’s sarcoma admitted to Pediatric Hematology and Oncology Department were evaluated retrospectively. The epidemiologic and clinical features, treatment results, long term side effects of therapy of 27 patients with Ewing’s sarcoma were analyzed. The mean age of patients with Ewing’s sarcoma (n=27) was 11.36 ±3.98 years (range,8 months-17.9 years) and male/female ratio was 1.7. The most common complaints were pain (96.3%) and swelling (100%). A total of 16 (59.3%) primary tumors were located in an extremity, whereas 11 (40.7%) occured in the axial skeleton. The most common primary lesion sites were femur (26%), tibia (14.8%), pelvis(14.8%) and fibula (11.1%) respectively. At diagnosis 3 patients (11.1%) had bone marrow metastases. A total of 9 patients (33.3%) had metastases at diagnosis. 23 patients (92%) received neoadjuvant chemotherapy and 17 patients received (77.2%) adjuvant chemotherapy. Limb salvage surgery was applied in 13 patients and amputation was made in 2 patients. Seventeen patients (65.3%) received radiotherapy; to the primary lesion site in 11 patients, the metastatic site in 5 patients, and both sites in one patient. The mean and median follow-up of patients with Ewing’s sarcoma was 35.5 and 19 months respectively (maximum 355 months). The overall survival rate was 22.4±10.9 % .

Kemoterapiye Hızlı Yanıt Veren Ewing Sarkomu’na Bağlı Spinal Kord Basısı Olgusu

Rapid Response to Chemotherapy in a Child with Spinal Cord Compression and Ewing Sarcoma Spinal-cord compression (SCC) is a critical oncologic emergency that must be recognized and treated early to increase the possibility of preventing permanent loss of function. Pain and deficits in motor, sensory, and autonomic function can occur. MRI and CT remain the two most useful diagnostic and management tools. Corticosteroids, radiation therapy, and surgery are the standard treatment options. We describe a patient with Ewing Sarcoma who p re s e n te d with SCC. He successfully treated with dexamethasone and chemotherapy. Spinal kord basisi (SKB) olusabilecek kalici fonksiyon kayiplarinin onlenmesi acisindan erken taninmasi ve tedavi edilmesi gereken onemli bir onkolojik acildir. Agri, duysal ve motor kayiplar, otonomik fonksiyon bozukluklari gorulebilir. MRG ve BT en kullanisli tanisal girisim araclaridir. Radyoterapi, cerrahi ve kortikosteroidler baslica tedavi secenekleridir. Burada kortikosteroid ve kemoterapi ile hizla duzelen, Ewing Sarkomu sonucu gelisen bir SKB olgusu sunulmaktadir.