Yavuz Köksal

Primary malignant skin tumors in children: Etiology, treatment and prognosis

Abstract Objective : The aim of the study was to evaluate the etiology, treatment and prognosis of the malignant skin tumors in children.

The efficacy of liver transplantation in malignant liver tumors associated with tyrosinemia: clinical and laboratory findings of five cases.

Abstract:  To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha‐fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living‐related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living‐related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living‐related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.

Comparison of accelerated and rapid schedules for monovalent hepatitis B and combined hepatitis A/B vaccines in children with cancer.

The aim of this study was to determine the efficacy of immunization against hepatitis A and B infections with “rapid” or “accelerated” schedules in children with cancer receiving chemotherapy. Fifty-one children were recruited to receive either vaccination schedule, in the “rapid vaccination schedule”; hepatitis B (group I) or combined hepatitis A/B vaccines (group III) were administered at months 0, 1, 2, and 12; in the “accelerated vaccination schedule,” hepatitis B (group II) or combined hepatitis A/B (group IV) vaccines were administered on days 0, 7, 21, and 365 intramuscularly. The seroconversion rates at months 1 and 3 were 35.7 and 57.1% in group I and 25 and 18.8% in group II, respectively. Group I developed higher seroconversion rates at month 3. In group III the seroconversion rates for hepatitis B at months 1 and 3 were 54.5 and 60% and in group IV 50 and 70%, respectively. For hepatitis A, the seroconversion rates at months 1 and 3 were 81.8 and 90% in group III and 80 and 88.9% in group IV, respectively. The accelerated vaccination schedule seems to have no advantage in children receiving cancer chemotherapy except for high antibody levels at month 1. In conclusion, the accelerated vaccination schedules are not good choices for cancer patients. The combined hepatitis A/B vaccine is more effective than monovalent vaccine in cancer patients, which probably can be explained by an adjuvant effect of the antigens. The seroconversion of hepatitis A by the combined hepatitis A/B vaccination is very good in cancer patients.

Mesenchymal hamartoma of the liver mimicking hepatoblastoma.

Mesenchymal hamartoma of the liver is a cystic benign liver mass occurring in children. Diagnostic confusion with hepatoblastoma may arise when alpha-feto-protein (AFP) level is elevated. We report an extremely rare case of mesenchymal hamartoma in an 11-month-old boy. Serum AFP was elevated and fine-needle aspiration biopsy suggested the lesion as hepatoblastoma, so he received preoperative chemotherapy. At the end of the preoperative chemotherapy, the tumor size and AFP level decreased. A right hepatectomy was performed. The pathologic examination of the specimen revealed mesenchymal hamartoma. Mesenchymal hamartoma of the liver with increased serum AFP levels may mimic hepatoblastoma if a cytological examination samples only the hepatocellular component of mesenchymal hamartoma. According to our knowledge, this is the first case of the mesenchymal hamartoma of the liver, which showed reduction in serum levels of AFP and involution of the tumor size by preoperative chemotherapy.

Merkel cell carcinoma in a child.

Merkel cell carcinoma, a rare tumor of the skin with aggressive behavior, is usually fatal when advanced disease is present. Merkel cell carcinoma occurs mostly in white race between 60 and 80 years of age, however, it can occur in any races and ages. It is extremely rare in children. We present here a Merkel cell carcinoma in a boy. Its features and treatment modalities were discussed in the literature light.

The treatment of childhood Hodgkin lymphoma: Improved survival in a developing country

Background. To evaluate the clinical characteristics, treatment regimens, and outcome of children with Hodgkin lymphoma in a developing country over a period of 34 years. Methods. This paper retrospectively evaluates the treatment and prognosis of 614 children with Hodgkin lymphoma disease between 1971 and 2005. All patients were treated with chemotherapy, and also received radiotherapy. Results. There were 452 males and 162 females with a median age of 8 years (2 to 21); 183 patients had B symptoms. There were 165, 185, 145, and 119 patients in stage I, II, III, and IV, respectively. Histopathologic subtypes were mixed cellularity (344 patients), nodular sclerosis (90), lymphocytic predominance (62), lymphocytic depletion (46), unclassified types (69), and nodular lymphocyte predominant Hodgkin lymphoma (3). Overall (OS) and event-free survival (EFS) rates were 83 and 60%, though OS rates varied according to chemotherapy protocol; age; presence of B symptoms, leukocytosis, anemia, and extranodal involvement; and stage at diagnosis. Over the years, the median age of patients increased, as did the frequency of the nodular sclerosing type of disease. Conclusions. This is one of the largest series in a single center. The increase in the median age and in the frequency of the nodular-sclerosing type are thought to be related to the development status of Turkey. The ABVD protocol yielded the best survival rates and should be used for treatment of patients with Hodgkin lymphoma.

Intracranial Desmoid Tumor with Familial Adenomatous Polyposis Coli

Intracranial desmoid tumors are extremely rare. The association of desmoid tumors with familial adenomatous polyposis coli was reported previously, with the tumors involving trunk and extremities. We report a 3.5-year-old girl with intracranial desmoid tumor with familial adenomatous polyposis coli. This condition in a child is rarely reported. Follow-up of the patient after cranial surgery and of the family for this premalignant inherited condition is necessary.

IMMUNOGENICITY OF HEPATITIS A VACCINE IN CHILDREN WITH CANCER

This study evaluated the immuned response of the hepatitis A vaccine in children with cancer who were receiving chemotherapy. Twenty-eight patients with lymphomas or solid tumors and who had negative serology for hepatitis A were enrolled. The median age was 4.7 years (range 2–16). The patients received 1440 IU hepatitis A vaccine at 0 and 6 months. Seroconversion rates at the first and seventh months were 60% (n = 17/28 patients) and 89% (n = 24/27 patients). No adverse effects were observed. The hepatitis A vaccine was found to be effective and safe in children with cancer.

Teratoid Wilms' tumor in a child

Teratoid Wilms’ tumor is a rare histopathological variant of Wilms’ tumor containing predominantly heterologous tissue: adipose, glial, muscular, cartilage, or bone. Until 1984 this type of histopathology was called renal teratoma. Variend et al. fi rst introduced the term in 1984 and to date several cases have been described in the literature. 1 The prognosis of patients with these tumors is better than that of patients with classic Wilms’ tumors. 1 – 13

Galactorrhea-Associated Granulosa Cell Tumor in a Child

Granulosa cell tumor of the ovary is a rare form of ovarian cancer in children. An 11-year-old girl was admitted with complaints of galactorrhea and abdominal mass. Abdomino-pelvic ultrasound and computed tomography revealed an ovarian tumor. Her prolactine and estradiol levels were increased but luteinizing hormone and follicle-stimulating hormone were decreased. An exploratory laparotomy revealed a giant solid mass, which was completely removed and determined as juvenile granulosa cell tumor. The clinical, hormonal, and radiological findings and the therapy of galactorrhea associated with granulosa cell tumor in a child are discussed. To our knowledge, this is first time it has been described in childhood.