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The paper describes the personal experience with one-stage operations for bilateral nephroblastoma (BN) in children.

Infantile fibrosarcoma (IF) is a rare soft tissue sarcoma that occurs with a frequency of 1% of all cases of malignant neoplasms in children in the first year of life. IF most often affects the soft tissues of the upper and lower extremities. Rare localizations include the trunk, the head and neck, and the gastrointestinal tract. The article describes a rare clinical case of congenital IF of soft tissues of the chest wall in a newborn, as well as a literature review.

Российский журнал ДЕТСКОЙ ГЕМАТОЛОГИИ и ОНКОЛОГИИ R u s s i a n J o u r n a l o f P e d i a t r i c H e m a t o l o g y а n d O n c o l o g y 48 О р и г и н а л ь н ы е и с с л е д о в а н и я || O r i g i n a l s t u d i e s

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland. Despite the low incidence of ACC the mortality rate ranges from 0.04 to 0.2 %, in the overall structure of cancer mortality. Treatment of ACC is mainly surgical and radical surgical excision is the treatment of choice for local disease stages.Aim of the study — to present our results of surgical treatment of localized and locally advanced ACC in children and to determine the risk factors of relapse.Materials and methods. Twenty-eight patients (median age of 47.8 (06—216) mo.) with localized and locally advanced ACC underwent a retrospectively analysis. Stage I, II, and III revealed in 12 (45 %), 7 (25 %), and 9 (30 %), respectively. In 19 (68 %) cases the secretion of one or more hormone observed. Macroscopically and microscopically complete resection were performed in 26 (93 %) and 23 (82 %) patients, respectively. The median tumor volume was 183 (3.6—1608) cm3 and the median tumor weight was 207.9 (48—710) g.Results. Five-year overall (OS) and relapse-free (RFS) survival were 71 % and 69 %, respectively. OS and RFS according to stage I, II, and III were 100 % vs. 71 % vs. 17 % and 100 % vs. 71 % vs. 14 % respectively. The radical surgical resection and the level of Ki-67 expression influenced significantly the rates of OS and RFS (p < 0.001).Conclusion. The main factor affecting the survival rate of ACC in children with stages I—III is the radical surgical resection. It should be taken into account when planning postoperative therapy. Some of biological characteristics of the tumor could also significantly affect the results of treatment.