Incidence and Trend of Childhood and Adolescent Cancers in Yazd, Iran.

BACKGROUND: Data on childhood and adolescent malignant tumors incidence are skimp in developing countries. In this study, we analyzed the incidence and trend of childhood and adolescent cancers in Yazd city, center of Iran between Jan 2004 and Dec 2013. MATERIAL AND METHODS: The various types of malignant tumors were grouped pursuant to the International Classification for Cancer in Children. To analyze the data, descriptive and illative statistical methods were used. RESULTS: Two hundred twenty-two patients with a malignancy aged less than 18 years were studied with a male to female ratio of 1.36.The mean age of patients was 9.88 (±5.7) years. Leukemia with the frequency of 84 (37.8%) and after that lymphoid malignancies with the frequency of 49 (22.1%) were the most common cancers. There was a low range of oscillation in the incidence rate of malignancies during this period of time (P value= 0.081). Malignancies were mostly in males (P value=0.057) but the whole process of malignancy incidence had gone toward the higher rate of incidence in females. Incidence rate of cancers types was steady. Malignancy incidence was 3-7 cases in hundred of thousands except a year of which this incidence rate was estimated 13.4. CONCLUSION: Leukemias and lymphomas were the main cancers in the center of Iran. Childhood and adolescent malignancies may be considerably under-recorded in our province .A childhood and adolescent cancer registry is necessary for exact analysis of these types of malignancies.

Results of combination chemotherapy and radiation therapy in non-metastatic gastric cancer in Yazd--Iran.

BACKGROUND: Gastric cancer is the first cause of cancer death in Iran. Most of the patients are diagnosed in advanced stages and survival rate is low. The aim of this study was to evaluate the survival rate of patients with gastric and gastroesophageal junction adenocarcinoma receiving combination chemotherapy and radiation therapy in Iran. MATERIALS AND METHODS: This study was conducted on 53 patients with gastric or gastroesophageal junction adenocarcinoma referring to Shahid Ramezanzadeh Radiation Oncology Center between 2004 and 2010. All patients underwent surgical operation, chemotherapy, and radiotherapy. Data contained in patients' records were extracted and follow-up was conducted through telephone contacts. To analyze the data, Kaplan Meier curves, and SPSS software were employed. RESULTS: Seventeen (32.1%) patients were female and 36 (67.9%) were male. Mean age was 58.32 years. In 14 patients (26.9%) no lymph node was resected or reported by the pathologist. In 29 patients (54.7%) one to six lymph nodes were found, and in10 patients (18.9%) seven lymph nodes or more were detected. In two patients, (3.77%) more than 15 lymph nodes were resected. Surgical staging was impossible for 16 patients (30.2%). Mean survival was 50.9 months and median survival was 51 months. 3,5 and 7 year survival rates were 73%, 36%, and 18% respectively. None of the variables had a significant relation to survival. CONCLUSIONS: Despite inadequate surgery, the survival rate in our study is one of the best one reported in our country. We think this advantage may be due to poly drug chemotherapy and sequential radiation therapy.

Clinicopathologic characteristics and outcome of childhood and adolescent Ewing's sarcoma in center of Iran.

BACKGROUND: Ewing's sarcoma family is a group of small round cells tumors. The aim of this study is to evaluate clinicopathologic characteristics and outcome of Ewing's sarcoma in children and adolescents in Yazd, Iran. MATERIALS AND METHODS: All patients under 19 years with documented pathology of Ewing's sarcoma family tumor who referred to Shahid Ramazanzadeh Radiotherapy center between 2002 to 2010 were enrolled in this retrospective study. Overall survival and disease free survival and prognostic factors were evaluated. RESULTS: Among approximately 80,000 patients who referred to Shahid Sadoughi pathology department, over an 8-year period, the total number of patients with Ewing sarcoma was 32, of which, 18 cases were under the age 19 . The mean age was 13.72 years. Five patients (27.8%) had metastatic disease at the time of diagnosis. Complete response had been achieved in 8 (44.4%) of the patients. Local recurrence occurred in 4 (22.2%) of the patients. During the follow up 13 (72.2%) of the patients showed metastases. The mean overall survival was 34.79 months (95% CI: 22.27-47.32) .One, two, four and five year survival was 72%, 39%, 25% and 17% respectively. Complete remission occurred in 10 patients (63.6%). A trend of better overall survival was found in these patients (p=0. 55). When the brain and bone metastases occurred, the overall survival decreased significantly (p=0. 003 ). CONCLUSIONS: The overall survival rate of Ewing's sarcoma is very low in comparison with other parts of the world.

Seroprovalence of herpes simplex1, 2 IgG antibodies in patients with beta thalassemia in a major tertiary care hospital located in Yazd, Iran.

BACKGROUND: Patients with beta thalassemia suffer from increased susceptibility to infections and putridity plays a major role in the patient's morbidity and mortality. The risk of transfusion-transmitted viral infection is well known in these patients. However, there is dearth of information about the seroprevalence of herpes simplex virus (HSV) infection in patients with beta thalassemia in literature. This study analyzes the prevalence of anti-HSV1, 2 IgG antibodies in patients with beta thalassemia in a major tertiary care hospital located in Yazd,Iran. MATERIAL AND METHODS: In this case control study, we undertook a serological study of HSV1,2 IgG antibodies among 45 patients with beta thalassemia and 45 healthy individuals as control group by ELISA method. A p.value <0.05 was considered statistically significant. Statistical analyses were performed using SPSS.20. RESULTS: The prevalence of HSV 1,2 IgG antibodies were estimated 88.8% among patients with beta thalassemia and 77.7% in control group. Regarding p.value=0.64, it showed no significant difference in these two groups. CONCLUSION: Although infectious diseases still represent a major challenge in patients with beta thalassemia, HSV past infection rate was not increased in these patients in our study. More studies are required to clarify this matter.

Gaucher's Disease, an Unusual Cause of Massive Splenomegaly, a Case Report.

BACKGROUND: Gaucher's Disease (G.D.) is an autosomal recessive disorder resulting from the accumulation of glucocerebrosidase in the cells of macrophage-monocyte system as a result of a deficiency in lysosomal glucocerebrosidase. This enzyme is encoded by a gene on chromosome-1. Here we report a case of Gaucher's Disease .G.D is rare in Yazd. CASE REPORTS: We reported a patient that presented with weakness, pallor and gradually increasing abdominal girth. Clinical examination and history pointed to be a lipid storage disease. Final diagnosis of G.D. was reported after examining the bone marrow smears. Confirmation of diagnosis on Gaucher's disease was performed by measurement of glucocerebrosidase level. CONCLUSION: We report a case of G.D. to emphasize the importance of early recognition by clinical manifestation and histological findings. G.D. should be considered in the differential diagnosis of children with unexplained splenomegaly.

Hematologic manifestations in a child with HIV; a Case Report.

BACKGROUND: Immune deficiency in human might be primary or secondary and could be seen with a wide variety of manifestations. In the following, we presented a Child with various complains that diagnosed to have HIV infection. CASE REPORT: A 2/5 y/o child was admitted to the hospital for FUO with prolonged cough, FTT, cervical lymphadenopathy, hepatosplenomegaly and bilateral optic neuritis. . He was hospitalized for fever, cytopenia and hepatosplenomegaly one year ago, and three months later in an outpatient visit, these signs improved, except thrombocytopenia. In evaluation, bicytopenia, elevated ESR, hyperlipidemia, hyperproteinemia, thrombosis of the transverse sinus of brain, antiphospholipid antibodies , decreased levels of protein S and factor V Leiden and increased level of anti thrombin III were detected. Consequently, the result of HIV antibody showed positive. In addition to warfarin and cotrimoxazole therapy, he was referred to special center for possible HARRT therapy. CONCLUSION: In approach to patients with various clinical presentations such as cytopenia, recurrent or persistent lymphadenopathy, unexplained hyperproteinemia or hyperlipidemia, evaluation of HIV infection is highly recommended for consideration and further therapy.

Tumefactive fibroinflammatory lesion: a rare aetiology for a neck mass in an old Iranian man.

Tumefactive fibroinflammatory lesion (TFIL) is a rare idiopathic, histologically benign appearing, tumour-forming fibrosclerosing lesion that clinically simulates a malignant neoplasm. There have been limited case reports of TFIL in foreign literature but none from Iran. Here, the authors report a case of TFIL in a 73-year-old Iranian man.

Ossifying fibromyxoid tumour: a rare soft tissue tumour of intermediate malignancy.

Ossifying fibromyxoid tumours (OFTs) are uncommon soft tissue tumours. A 73-year-old male presented with a painless exophytic mass located in the left scapular area which was identified 1 year earlier. An incisional biopsy was performed and microscopic features were compatible with OFT. Radiologic evaluation revealed multiple pulmonary metastasis. Although many cases persue an indolent biologic behaviour, local recurrence and metastasis have been reported and long-term follow-up should be considered.

Focal myositis of the thigh misdiagnosed radiologically as rhabdomyosarcoma.

Focal myositis is a commonly referenced, infrequently reported and poorly documented benign inflammatory pseudotumour, which may be misdiagnosed as a malignant tumour. We report the clinicopathologic features in a case of focal myositis in the thigh of a 15-year-old boy.

Nevoid malignant melanoma in an albino woman.

Albinism is a disorder of hypopigmentation affecting the skin, hair and eyes. Ultraviolet light induced cutaneous tumours are common in patients with albinism due to reduced or absent protection from melanin, with squamous cell carcinoma being the most common. Although non-melanomatous skin cancers are more frequent in patients with albinism, dysplastic nevi and melanoma present a greater diagnostic challenge in this group because of their hypopigmented appearance. Here the authors report a case of nevoid malignant melanoma in a patient who had type 1A oculocutaneous albinism.